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6. Wang XW: [Pathogenesis and management of hemorrhage and thrombosis in plasma cell dyscrasias]. Zhongguo Shi Yan Xue Ye Xue Za Zhi; 2010 Jun;18(3):809-15
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  • [Title] [Pathogenesis and management of hemorrhage and thrombosis in plasma cell dyscrasias].
  • Unexpectedly high rates of venous thromboembolic events (VTE) induced by highly effective immune modulating drugs thalidomide and lenalidomide for treatment of multiple myeloma have focused attention on the incidence and underlying pathophysiology of VTE in patients with plasma cell dyscrasias, and on thromboprophylaxis approaches.
  • While bleeding complications are relatively uncommon in the patients with lymphoproliferative disorders, acquired von Willebrand syndrome, typically occurring in the patients with monoclonal gammopathy of unknown significance, and acquired coagulopathies associated with primary amyloidosis can present with haemorrhagic complications and both are challenges to the management.
  • This review highlights these important haemostasis-related complications of plasma cell dyscrasias and provides an overview of other uncommon bleeding and thrombotic events that can affect diagnosis and therapeutic management of clonal plasma cell disorders.

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  • (PMID = 20561456.001).
  • [ISSN] 1009-2137
  • [Journal-full-title] Zhongguo shi yan xue ye xue za zhi
  • [ISO-abbreviation] Zhongguo Shi Yan Xue Ye Xue Za Zhi
  • [Language] CHI
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] China
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7. Garcia T, Dafer R, Hocker S, Schneck M, Barton K, Biller J: Recurrent strokes in two patients with POEMS syndrome and Castleman's disease. J Stroke Cerebrovasc Dis; 2007 Nov-Dec;16(6):278-84
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  • [Title] Recurrent strokes in two patients with POEMS syndrome and Castleman's disease.
  • POEMS syndrome is a disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes.
  • Castleman's disease is a rare lymphoproliferative disorder that can be present in patients with POEMS syndrome.
  • However, only 3 patients have ever been described with POEMS syndrome, Castleman's disease, and stroke.
  • We suspect that the strokes are a result of a possible intracranial vasculopathic process.
  • [MeSH-major] Brain Ischemia / complications. Giant Lymph Node Hyperplasia / diagnosis. POEMS Syndrome / diagnosis. Stroke / etiology

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  • (PMID = 18035247.001).
  • [ISSN] 1532-8511
  • [Journal-full-title] Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association
  • [ISO-abbreviation] J Stroke Cerebrovasc Dis
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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8. Bochtler T, Hegenbart U, Heiss C, Benner A, Cremer F, Volkmann M, Ludwig J, Perz JB, Ho AD, Goldschmidt H, Schonland SO: Evaluation of the serum-free light chain test in untreated patients with AL amyloidosis. Haematologica; 2008 Mar;93(3):459-62
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  • The FLC test detected the monoclonal gammopathy in 87% compared with 92% for immunofixation of serum and urine in combination.
  • Higher FLC levels were associated with higher bone marrow plasmocytosis, poorer Karnofsky index and heart involvement, and therefore reflected disease severity.
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Bone Marrow / pathology. Female. Humans. Karnofsky Performance Status. Kidney / pathology. Kidney Failure, Chronic / blood. Kidney Failure, Chronic / etiology. Kidney Failure, Chronic / pathology. Kidney Failure, Chronic / urine. Male. Middle Aged. Myocardium / pathology. Nephelometry and Turbidimetry. Plasma Cells / pathology. Severity of Illness Index

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  • (PMID = 18287137.001).
  • [ISSN] 1592-8721
  • [Journal-full-title] Haematologica
  • [ISO-abbreviation] Haematologica
  • [Language] eng
  • [Publication-type] Clinical Trial; Comparative Study; Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Amyloid; 0 / Immunoglobulin Light Chains
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9. Landgren O, Gridley G, Turesson I, Caporaso NE, Goldin LR, Baris D, Fears TR, Hoover RN, Linet MS: Risk of monoclonal gammopathy of undetermined significance (MGUS) and subsequent multiple myeloma among African American and white veterans in the United States. Blood; 2006 Feb 1;107(3):904-6
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  • [Title] Risk of monoclonal gammopathy of undetermined significance (MGUS) and subsequent multiple myeloma among African American and white veterans in the United States.
  • A few small studies have reported a higher prevalence of monoclonal gammopathy of undetermined significance (MGUS) in African Americans versus whites.
  • Etiologic factors for MGUS and determinants for transformation of MGUS to MM are unknown.
  • We quantified the prevalence of MGUS and subsequent risk of MM among 4 million African American and white male veterans admitted to Veterans Affairs (VA) hospitals.
  • The age-adjusted prevalence ratio of MGUS in African Americans compared with whites was 3.0 (2.7-3.3 95% confidence interval).
  • Among 2046 MGUS cases, the estimated cumulative risk of MM during the first 10 years of follow-up was similar (P = .37) for African Americans (17%) and whites (15%).
  • In the largest study to date, we suggest that the excess risk of MM in African Americans results from an increase in risk of MGUS rather than an increased risk of progression from MGUS to MM.

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  • [Cites] N Engl J Med. 2002 Feb 21;346(8):564-9 [11856795.001]
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  • (PMID = 16210333.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] ENG
  • [Grant] United States / Intramural NIH HHS / /
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Intramural
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC1895893
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10. Muslimani AA, Spiro TP, Chaudhry AA, Taylor HC, Jaiyesimi I, Daw HA: Venous thromboembolism in patients with monoclonal gammopathy of undetermined significance. Clin Adv Hematol Oncol; 2009 Dec;7(12):827-32
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  • [Title] Venous thromboembolism in patients with monoclonal gammopathy of undetermined significance.
  • Monoclonal gammopathy of undetermined significance (MGUS) is defined by the presence of a serum M-protein at a concentration of 3 g/dL or less, with less than 10% plasma cells in the bone marrow, and the absence of lytic bone lesions, anemia, hypercalcemia, and renal insufficiency related to the plasma cell proliferative process.
  • The annual risk of MGUS progressing to a symptomatic plasma cell proliferation or other related malignancy is approximately 1%.
  • In this retrospective study of MGUS patients, VTE was seen in 8% (9/112) of patients, a rate that is 22.8-fold higher than that in the general population (P is less than .001).
  • Although many studies have identified VTE as a marker for subsequent malignancy, we did not find a significant difference in the incidence of VTE as a function of the risk factor group.
  • [MeSH-major] Monoclonal Gammopathy of Undetermined Significance / complications. Venous Thromboembolism / epidemiology
  • [MeSH-minor] Disease Progression. Humans. Retrospective Studies. Risk Assessment. Survival Rate

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  • (PMID = 20332755.001).
  • [ISSN] 1543-0790
  • [Journal-full-title] Clinical advances in hematology & oncology : H&O
  • [ISO-abbreviation] Clin Adv Hematol Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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11. Decaux O, Ruelland A, Grosbois B: Effect of treatment on biclonal gammopathy associated with Gaucher disease. J Inherit Metab Dis; 2007 Oct;30(5):824
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  • [Title] Effect of treatment on biclonal gammopathy associated with Gaucher disease.
  • The non-random association of Gaucher disease with polyclonal and monoclonal gammopathy has been known since 1950.
  • The effect of treatment on monoclonal gammopathy is not well documented.
  • We report on the long-term evolution of a biclonal gammopathy in a patient with type I Gaucher disease who was treated with splenectomy and enzyme replacement therapy.
  • Immunofixation identified two monoclonal components: IgG kappa and IgA kappa.
  • Our observation and previous reports suggest that enzyme replacement therapy is more effective in polyclonal hypergammaglobulinaemia than in monoclonal gammopathy.
  • [MeSH-major] Gaucher Disease / drug therapy. Glucosylceramidase / therapeutic use. Paraproteinemias / etiology. Splenectomy. gamma-Globulins / metabolism

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  • (PMID = 17879146.001).
  • [ISSN] 1573-2665
  • [Journal-full-title] Journal of inherited metabolic disease
  • [ISO-abbreviation] J. Inherit. Metab. Dis.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Recombinant Proteins; 0 / gamma-Globulins; EC 3.2.1.- / Hexosaminidases; EC 3.2.1.- / chitotriosidase; EC 3.2.1.45 / Glucosylceramidase; EC 3.2.1.45 / imiglucerase
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12. Ito Y, Nishimura K, Yamanaka K, Hakamada A, Isoda K, Kurokawa I, Mizutani H: Necrobiotic xanthogranuloma with paraproteinemia; an atypical case. J Dtsch Dermatol Ges; 2008 Jan;6(1):40-3
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  • Biopsies showed a non-palisading granuloma with numerous multinucleated giant cells showing prominent elastophagocytosis and extensive areas of necrobiosis throughout the entire dermis.
  • Immunohistochemical staining detected the presence of roughly equal numbers of IgG-lambda-and IgG-kappa-staining plasma cells in the dermis.
  • We diagnosed NXG with paraproteinemia with monoclonal gammopathy (IgG-lambda type) of unknown significance.

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  • (PMID = 17941882.001).
  • [ISSN] 1610-0387
  • [Journal-full-title] Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
  • [ISO-abbreviation] J Dtsch Dermatol Ges
  • [Language] eng; ger
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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13. Pérez-Andrés M, Almeida J, Martín-Ayuso M, Moro MJ, Martín-Nuñez G, Galende J, Borrego D, Rodríguez MJ, Ortega F, Hernandez J, Moreno I, Domínguez M, Mateo G, San Miguel JF, Orfao A, Spanish Network on multiple myeloma (G03/136), Spanish Network of Cancer Research Centers (C03/10): Clonal plasma cells from monoclonal gammopathy of undetermined significance, multiple myeloma and plasma cell leukemia show different expression profiles of molecules involved in the interaction with the immunological bone marrow microenvironment. Leukemia; 2005 Mar;19(3):449-55
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  • [Title] Clonal plasma cells from monoclonal gammopathy of undetermined significance, multiple myeloma and plasma cell leukemia show different expression profiles of molecules involved in the interaction with the immunological bone marrow microenvironment.
  • The immunological bone marrow (BM) microenvironment plays a major role in controlling growth and survival of clonal plasma cells (PC); this might translate into different patterns of expression of molecules involved in immune responses on PC from different types of monoclonal gammopathies (MG).
  • We have studied the expression of a group of nine such molecules on both BMPC and the plasma of 61 newly diagnosed MG patients (30 MG of undetermined significance (MGUS), 27 multiple myeloma (MM) and four plasma cell leukemia (PCL)) and five normal individuals.
  • Clonal PC from all MG displayed significantly increased levels of CD56, CD86 and CD126, and decreased amounts of CD38 (P<0.001).
  • Additionally, HLA-I and beta2-microglobulin were abnormally highly expressed in MGUS, while CD40 expression was decreased in MM and PCL (P<0.05).
  • Interestingly, a progressive increase in the soluble levels of beta2-microglobulin was found from MGUS to MM and PCL patients (P=0.03).
  • Overall, those phenotypic differences are consistent with increased antigen presentation and costimulatory capacities in MGUS, which progressively deteriorate in malignant MG (MM and PCL).
  • [MeSH-major] Antigens, CD / genetics. Bone Marrow / pathology. Leukemia, Plasma Cell / genetics. Multiple Myeloma / genetics. Paraproteinemias / immunology. Plasma Cells / immunology


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4. Chim CS, Liang R, Leung MH, Kwong YL: Aberrant gene methylation implicated in the progression of monoclonal gammopathy of undetermined significance to multiple myeloma. J Clin Pathol; 2007 Jan;60(1):104-6
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  • [Title] Aberrant gene methylation implicated in the progression of monoclonal gammopathy of undetermined significance to multiple myeloma.
  • Malignant transformation is a multistep process that may involve dysregulation of oncogenes and tumour suppressor genes, and monoclonal gammopathy of undetermined significance (MGUS) is believed to be a precursor of multiple myeloma.
  • To investigate whether aberrant promoter methylation might be involved in the evolution of MGUS to multiple myeloma, we examined the p16, protein tyrosine phosphatase, non-receptor type 6 (SHP1), death-associated protein (DAP) kinase, E-cadherin and oestrogen receptor genes, most being tumour suppressor genes, by methylation-specific polymerase chain reaction.
  • In 32 cases of multiple myeloma and 19 cases of MGUS, significantly more frequent methylation of p16 (p = 0.001), SHP1 (p< or =0.001) and E-cadherin (p< or =0.001) genes was found in multiple myeloma than in MGUS.
  • Methylation of DAP kinase and oestrogen receptor genes was comparable in multiple myeloma and MGUS.
  • In conclusion, methylation of p16, SHP1 and E-cadherin genes might be involved in the progression of MGUS to multiple myeloma.
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. DNA, Neoplasm / genetics. Disease Progression. Female. Genes, Tumor Suppressor. Humans. Male. Middle Aged. Multiple Myeloma / genetics. Neoplasm Proteins / genetics. Polymerase Chain Reaction / methods. Promoter Regions, Genetic / genetics


15. Hirano T, Hoshino Y: Detection of changes in the nuclear phase and evaluation of male germ units by flow cytometry during in vitro pollen tube growth in Alstroemeria aurea. J Plant Res; 2009 Mar;122(2):225-34
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  • Sperm cell formation occurred at 6-9 h after culture, indicating that the first change was due to the division of the generative cells into sperm cells.
  • After sperm cell formation, the number of vegetative nucleus associations with sperm cells showed a tendency to increase.
  • When sperm cells, vegetative nuclei, and partial MGUs were collected separately from pollen tubes cultured for 18 h and analyzed using a flow cytometer, the sperm cells and vegetative nuclei contained 1C DNA, while the DNA content of partial MGUs was counted as 2C.
  • Therefore, the second change in the nuclear phase, which results in an increase in 2C nuclei, is possibly related to the formation of MGUs.
  • [MeSH-major] Alstroemeria / genetics. Cell Nucleus / metabolism. Flow Cytometry / methods. Pollen / cytology. Pollen / metabolism. Pollen Tube / growth & development
  • [MeSH-minor] Cell Differentiation. DNA, Plant / chemistry. Genes, Plant

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  • (PMID = 19151916.001).
  • [ISSN] 1618-0860
  • [Journal-full-title] Journal of plant research
  • [ISO-abbreviation] J. Plant Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / DNA, Plant
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16. Kaiser M, Mieth M, Liebisch P, Oberländer R, Rademacher J, Jakob C, Kleeberg L, Fleissner C, Braendle E, Peters M, Stover D, Sezer O, Heider U: Serum concentrations of DKK-1 correlate with the extent of bone disease in patients with multiple myeloma. Eur J Haematol; 2008 Jun;80(6):490-4
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  • [Title] Serum concentrations of DKK-1 correlate with the extent of bone disease in patients with multiple myeloma.
  • OBJECTIVES: Lytic bone disease is a hallmark of multiple myeloma (MM) and is caused by osteoclast activation and osteoblast inhibition.
  • METHODS: DKK-1 serum levels were quantified in 184 untreated MM patients and 33 monoclonal gammopathy of undetermined significance (MGUS) patients by ELISA, using a monoclonal anti-DKK-1 antibody.
  • RESULTS: Serum DKK-1 was elevated in MM as compared with MGUS (mean 11 963 pg/mL vs. 1993 pg/mL; P < 0.05).
  • Importantly, myeloma patients without lytic lesions in conventional radiography had significantly lower DKK-1 levels than patients with lytic bone disease (mean 3114 pg/mL vs. 17 915 pg/mL; P = 0.003).
  • CONCLUSION: Using a large series of myeloma patients, we could show for the first time a correlation between DKK-1 serum concentration and the amount of lytic bone disease, indicating that DKK-1 is an important factor for the extent of bone disease and supporting the hypothesis of DKK-1 as a therapeutic target in myeloma bone disease.

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  • (PMID = 18331598.001).
  • [ISSN] 1600-0609
  • [Journal-full-title] European journal of haematology
  • [ISO-abbreviation] Eur. J. Haematol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / DKK1 protein, human; 0 / Intercellular Signaling Peptides and Proteins
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17. Decaux O, Rodon P, Ruelland A, Estepa L, Leblay R, Grosbois B: [Epidemiology of monoclonal gammopathy in a general hospital and a university internal medicine department]. Rev Med Interne; 2007 Oct;28(10):670-6
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  • [Title] [Epidemiology of monoclonal gammopathy in a general hospital and a university internal medicine department].
  • [Transliterated title] Epidémiologie descriptive des gammapathies monoclonales. Expérience d'un centre hospitalier général et d'un service de médecine interne de centre hospitalier et universitaire.
  • PURPOSE: Monoclonal gammopathy are common in the general population.
  • We describe biological features and etiology of monoclonal gammopathy diagnosed during more than a ten year period in the Internal Medicine Department of Rennes University Hospital and in all the medical departments of General Hospital of Blois.
  • RESULTS: Internal Medicine Department of Rennes University Hospital: 1051 monoclonal gammapathies were identified: 514 men and 537 women.
  • Isotypes repartition was: IgG 42.8% (450 cases), IgM 31.9% (335), IgA 8.9% (94) biclonal gammopathy 9.8% (103).
  • Sixty-nine monoclonal light chains (6.6%) were identified.
  • Median concentration of monoclonal protein was 14 g/l (1.8-104.4).
  • All department of General Hospital of Blois: 1282 monoclonal gammapathies were identified: 700 men and 582 women.
  • Thirty-four monoclonal light chains (2.7%) were identified.
  • Median concentration of monoclonal protein was 5.6 g/l (0.5-96.6).
  • Most frequent diagnosis were: monoclonal gammopathy of undetermined significance or MGUS (77.6% in Blois and 64.1% in Rennes), multiple myeloma (11.9% and 12.7%), Waldenström's macroglobulinemia (4.4% and 8.7%).
  • CONCLUSION: Monoclonal gammopathy are common in clinical practice.
  • MGUS account for more than 60% of monoclonal gammopathy.

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  • (PMID = 17566610.001).
  • [ISSN] 0248-8663
  • [Journal-full-title] La Revue de medecine interne
  • [ISO-abbreviation] Rev Med Interne
  • [Language] FRE
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Immunoglobulin A; 0 / Immunoglobulin G; 0 / Immunoglobulin Isotypes; 0 / Immunoglobulin Light Chains; 0 / Immunoglobulin M
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18. Wood AJ, Wagner MV, Abbott JJ, Gibson LE: Necrobiotic xanthogranuloma: a review of 17 cases with emphasis on clinical and pathologic correlation. Arch Dermatol; 2009 Mar;145(3):279-84
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • OBJECTIVE: To identify correlations between clinical presentation, specific histopathologic findings, and subsequent disease course in patients with necrobiotic xanthogranuloma (NXG).
  • PATIENTS: Seventeen patients with a diagnosis of NXG established between January 1, 1994, and December 31, 2007.
  • MAIN OUTCOME MEASURES: Description and distribution of clinical lesions, presence of monoclonal gammopathy, multiple myeloma, and correlation with microscopic patterns of skin lesions.
  • Twelve patients (71%) had a monoclonal gammopathy; of these, 3 (18%) had multiple myeloma.
  • Histopathologic examination of 12 patients showed findings consistent with NXG, including a bandlike pattern of necrobiotic granulomatous inflammation, atypical giant cells, cholesterol clefts, and plasma cells.
  • Although most patients had a serum monoclonal gammopathy, staining with antibodies to CD3, CD20, kappa light chains, and lambda light chains showed polytypic lymphocytes and plasma cells in all cases.
  • However, the skin lesions in NXG represent reactive inflammation and are not associated with the presence of monoclonal plasma cells or multiple myeloma.

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  • (PMID = 19289757.001).
  • [ISSN] 1538-3652
  • [Journal-full-title] Archives of dermatology
  • [ISO-abbreviation] Arch Dermatol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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19. Heider U, Kaiser M, Mieth M, Lamottke B, Rademacher J, Jakob C, Braendle E, Stover D, Sezer O: Serum concentrations of DKK-1 decrease in patients with multiple myeloma responding to anti-myeloma treatment. Eur J Haematol; 2009 Jan;82(1):31-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • In this study, the effect of different treatment regimens for MM on serum DKK-1 was evaluated and correlated with the response to treatment in 101 myeloma patients receiving bortezomib, thalidomide, lenalidomide, adriamycin and dexamethasone (AD) or high-dose chemotherapy (HDCT) followed by autologous stem cell transplantation (ASCT).
  • At baseline, myeloma patients had increased serum DKK-1 as compared with patients with MGUS (mean 3786 pg/mL vs. 1993 pg/mL).
  • Thalidomide led to a non-significant decrease in DKK-1 (1705 pg/mL vs. 1269 pg/mL, P = 0.081).
  • Within all groups, a significant decrease of DKK-1 was only seen in responders (i.e. patients achieving complete remission or partial remission), but not in non-responders.

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  • (PMID = 19067746.001).
  • [ISSN] 1600-0609
  • [Journal-full-title] European journal of haematology
  • [ISO-abbreviation] Eur. J. Haematol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / DKK1 protein, human; 0 / Intercellular Signaling Peptides and Proteins
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20. Fain O: [Monoclonal gammopathies]. Rev Prat; 2006 Jan 15;56(1):40-50
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Monoclonal gammopathies].
  • [Transliterated title] Immunoglobulines monoclonales pathogènes.
  • Monoclonal gammopathies can induce clinical or biological symptoms, independent of any associated hemopathy.
  • Peripheral neuropathies associated with monoclonal gammopathy are chronic, distal, symmetrical and progressive, in two thirds of the cases the monoclonal component reacts with a neuronal antigen principally myelin associated glycoprotein.
  • POEMS syndrome is characterized by: polyneuropathy, organomegaly, endocrine disorder, monoclonal component, and skin diseases.
  • Other manifestations are described: angioneurotic oedema, acquired Willebrand disease, systemic capillary leak syndrome, Fanconi syndrome.
  • Treatment of the gammopathy can control associated affections.

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  • (PMID = 16548248.001).
  • [ISSN] 0035-2640
  • [Journal-full-title] La Revue du praticien
  • [ISO-abbreviation] Rev Prat
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Number-of-references] 34
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21. Stankowski-Drengler T, Gertz MA, Katzmann JA, Lacy MQ, Kumar S, Leung N, Hayman SR, Buadi F, Kyle RA, Rajkumar SV, Dispenzieri A: Serum immunoglobulin free light chain measurements and heavy chain isotype usage provide insight into disease biology in patients with POEMS syndrome. Am J Hematol; 2010 Jun;85(6):431-4
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  • [Title] Serum immunoglobulin free light chain measurements and heavy chain isotype usage provide insight into disease biology in patients with POEMS syndrome.
  • POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes) syndrome is a rare paraneoplastic syndrome in which nearly all patients have a monoclonal lambda restricted plasma cell disorder.
  • Cystatin C levels were measured to discern whether subclinical renal insufficiency could account for FLC elevations in the presence of a normal FLC ratio.
  • The rise in serum FLC of POEMS patients appeared to be multifactorial-both a function of subclinical renal insufficiency and polyclonal activation of medullary and extramedullary plasma cells.
  • In summary, serum immunoglobulin profiles are unique in POEMS syndrome as compared with other plasma cell disorders.

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  • [Copyright] 2010 Wiley-Liss, Inc.
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  • (PMID = 20513121.001).
  • [ISSN] 1096-8652
  • [Journal-full-title] American journal of hematology
  • [ISO-abbreviation] Am. J. Hematol.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA125614-04; United States / NCI NIH HHS / CA / R01 CA125614-04; United States / NCI NIH HHS / CA / P01 CA062242; United States / NCI NIH HHS / CA / R01 CA125614; United States / NCI NIH HHS / CA / CA62242
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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  • [Other-IDs] NLM/ NIHMS213530; NLM/ PMC2902776
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22. Quartarone E, Alonci A, Allegra A, Bellomo G, Calabrò L, D'Angelo A, Del Fabro V, Grasso A, Cincotta M, Musolino C: Differential levels of soluble angiopoietin-2 and Tie-2 in patients with haematological malignancies. Eur J Haematol; 2006 Dec;77(6):480-5
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  • The system involving angiopoietin-2 (Ang-2) and its receptor, Tie-2, appears to play an important role not only in tumor angiogenesis, but also in the biology of haematological and non-haematological malignancies.
  • Measurements were carried out in 15 patients with chronic myeloid leukaemia (CML), 25 with essential thrombocythemia (ET), 24 with multiple myeloma (MM) and six with monoclonal gammopathy of undetermined significance (MGUS).
  • In patients with CML and MM the levels of sAng-2 were significantly higher (1686.53 +/- 936.41 pg/mL and 1917.82 +/- 1427 pg/mL, respectively) than in controls (n = 15; 996.096 +/- 414.65 pg/mL) (P < 0.01).
  • In patients with MM sAng-2 levels were significantly increased with increasing stage of disease, from stage I to stage III (P < 0.03) and presented a trend of correlation with Beta2-microglobulin levels (r = 0.317) and grade of bone involvement.
  • Furthermore, the levels of sAng-2 determined after 6 months of chemotherapy in CML patients were significantly lower than at diagnosis in the patients who achieved haematological remission.
  • Circulating sTie-2 levels were increased in patients with ET (17.5 +/- 9.2 vs 9 +/- 3.5 ng/mL; P < 0.01) and in those with CML (16.29 +/- 8.7 ng/mL; P < 0.04).
  • [MeSH-minor] Aged. Disease Progression. Female. Humans. Leukemia, Myelogenous, Chronic, BCR-ABL Positive / genetics. Leukemia, Myelogenous, Chronic, BCR-ABL Positive / metabolism. Male. Middle Aged. Multiple Myeloma / genetics. Multiple Myeloma / metabolism. Neovascularization, Pathologic. Paraproteinemias / genetics. Paraproteinemias / metabolism. Thrombocythemia, Essential / genetics. Thrombocythemia, Essential / metabolism

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  • (PMID = 16978237.001).
  • [ISSN] 0902-4441
  • [Journal-full-title] European journal of haematology
  • [ISO-abbreviation] Eur. J. Haematol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Angiopoietin-2; EC 2.7.10.1 / Receptor, TIE-2
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23. Saito N, Konishi K, Ohta S, Kondo T, Kato M, Hashino S, Takeda H, Asaka M, Ooi HK: Plural light chains in a single plasma cell of a monoclonal gammopathy undetermined significance case: an ultrastructural study. Hum Cell; 2007 Feb;20(1):10-4
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  • [Title] Plural light chains in a single plasma cell of a monoclonal gammopathy undetermined significance case: an ultrastructural study.
  • Bone marrow examination showed normal cellular marrow containing 6.3% of plasma cells with no abnormal features.
  • No chromosomal abnormality was observed at all.
  • The patient was diagnosed as having monoclonal gammopathy of undetermined significance.
  • The bone marrow plasma cells possessed free kappa- and lambda-chains in Golgi apparatus, rough endoplasmic reticula and cytoplasmic matrices.
  • Plural light chains were simultaneously produced with the same heavy chain in a plasma cell by immunoelectron microscopy.
  • This is the first report in the world of a monoclonal gammopathy of undetermined significance producing plural light chains with the same heavy chain.
  • [MeSH-major] Immunoglobulin Light Chains / analysis. Monoclonal Gammopathy of Undetermined Significance / immunology. Plasma Cells / immunology

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  • (PMID = 17506772.001).
  • [ISSN] 0914-7470
  • [Journal-full-title] Human cell
  • [ISO-abbreviation] Hum. Cell
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Immunoglobulin G; 0 / Immunoglobulin Light Chains; 0 / Myeloma Proteins
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24. Scavelli C, Nico B, Cirulli T, Ria R, Di Pietro G, Mangieri D, Bacigalupo A, Mangialardi G, Coluccia AM, Caravita T, Molica S, Ribatti D, Dammacco F, Vacca A: Vasculogenic mimicry by bone marrow macrophages in patients with multiple myeloma. Oncogene; 2008 Jan 24;27(5):663-74
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  • Bone marrow macrophages of patients with active and nonactive multiple myeloma (MM), monoclonal gammopathies of undetermined significance (MGUS) and benign anemia (controls) were stimulated for 7 days with vascular endothelial growth factor (VEGF) and basic fibroblast growth factor (bFGF), and analysed for the expression of endothelial cell (EC) markers by reverse transcription (RT)-PCR, real-time RT-PCR, western blot and immunofluorescence.
  • Active MM macrophages exposed to VEGF and bFGF acquired EC markers and formed capillary-like structures mimicking paired bone marrow ECs (multiple myeloma patient-derived endothelial cells, MMECs), with major responsiveness compared to macrophages from nonactive MM, MGUS or controls.
  • These figures were rare in nonactive MM and absent in MGUS or controls.
  • Our data indicate that macrophages contribute to build neovessels in active MM through vasculogenic mimicry, and this ability proceeds parallel to progression of the plasma cell tumors.
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Anemia / physiopathology. Bone Marrow Cells. Case-Control Studies. Cell Culture Techniques. Disease Progression. Female. Fibroblast Growth Factor 2 / physiology. Humans. Male. Middle Aged. Paraproteinemias / physiopathology. Reverse Transcriptase Polymerase Chain Reaction. Vascular Endothelial Growth Factor A / physiology

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  • (PMID = 17667938.001).
  • [ISSN] 1476-5594
  • [Journal-full-title] Oncogene
  • [ISO-abbreviation] Oncogene
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Vascular Endothelial Growth Factor A; 103107-01-3 / Fibroblast Growth Factor 2
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25. Sasaki H, Kato T, Murakami A, Nakayasu K, Yajima Y: [A case of monoclonal gammopathy with corneal stroma deposits]. Nippon Ganka Gakkai Zasshi; 2006 Apr;110(4):307-11
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  • [Title] [A case of monoclonal gammopathy with corneal stroma deposits].
  • BACKGROUND: Monoclonal gammopathy is a group of disorders characterized by proliferation of a single clone of plasma cells that produce monoclonal protein.
  • Sometimes benign monoclonal gammopathy that is a symptomatic can turn into a malignancy like multiple myeloma.
  • We present a case of monoclonal gammopathy with corneal deposits which was treated with deep lamellar keratoplasty (DLKP).
  • RESULTS: We diagnosed monoclonal gammopathy in a blood test that showed elevation of serum immunoglobulin G and the Kappa chain.
  • CONCLUSIONS: Corneal deposits of unknown origin might turn into monoclonal gammopathy that could be a life-threatening disease.
  • It is important for ophthalmologists to check the whole body of a patient when finding corneal deposits.
  • [MeSH-major] Corneal Diseases / complications. Corneal Stroma / pathology. Monoclonal Gammopathy of Undetermined Significance / complications

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  • (PMID = 16642949.001).
  • [ISSN] 0029-0203
  • [Journal-full-title] Nippon Ganka Gakkai zasshi
  • [ISO-abbreviation] Nippon Ganka Gakkai Zasshi
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
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26. Schilling G, Dierlamm J, Hossfeld DK: Prognostic impact of cytogenetic aberrations in patients with multiple myeloma or monoclonal gammopathy of unknown significance. Hematol Oncol; 2005 Sep-Dec;23(3-4):102-7
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  • [Title] Prognostic impact of cytogenetic aberrations in patients with multiple myeloma or monoclonal gammopathy of unknown significance.
  • Although the yield of metaphases due to a low proliferation rate is considerably lower in plasma cell dyscrasias, a number of chromosomal changes with prognostic implications have been identified in the past years, particularly due to the introduction of new techniques.
  • This article gives a short survey of the most important genetic alterations and their prognostic influence on the outcome of patients with plasma cell malignancies known to date.
  • [MeSH-minor] Humans. Karyotyping. Leukemia / genetics. Leukemia / pathology. Leukemia / therapy. Metaphase. Plasma Cells / pathology. Prognosis. Treatment Outcome

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  • [Copyright] 2005 John Wiley & Sons, Ltd.
  • (PMID = 16342296.001).
  • [ISSN] 0278-0232
  • [Journal-full-title] Hematological oncology
  • [ISO-abbreviation] Hematol Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 59
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27. Pérez-Persona E, Mateo G, García-Sanz R, Mateos MV, de Las Heras N, de Coca AG, Hernández JM, Galende J, Martín-Nuñez G, Bárez A, Alonso JM, Martín A, López-Berges C, Orfao A, San Miguel JF, Vidriales MB: Risk of progression in smouldering myeloma and monoclonal gammopathies of unknown significance: comparative analysis of the evolution of monoclonal component and multiparameter flow cytometry of bone marrow plasma cells. Br J Haematol; 2010 Jan;148(1):110-4
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  • [Title] Risk of progression in smouldering myeloma and monoclonal gammopathies of unknown significance: comparative analysis of the evolution of monoclonal component and multiparameter flow cytometry of bone marrow plasma cells.
  • The present study explored the impact of two novel criteria; having >95% abnormal plasma cells by flow cytometry at diagnosis and the evolving subtype of the disease, as predictors of progression in 61 smouldering multiple myeloma (SMM) and 311 monoclonal gammopathy of unknown significance (MGUS) patients.
  • Although both criteria were of prognostic value, the risk of progression was better identified by immunophenotyping [Hazard Ratio (HR) 6.2 and 17.2 for SMM and MGUS, respectively] than evolving subtype, which had independent prognostic value only in MGUS (HR 3.6).
  • Immunophenotyping discriminated the different risk of progression within the evolving and non-evolving subgroups of SMM (P = 0.01) and MGUS (P < 0.001).
  • [MeSH-major] Monoclonal Gammopathy of Undetermined Significance / immunology. Multiple Myeloma / immunology. Plasma Cells / immunology
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Disease Progression. Female. Flow Cytometry / methods. Humans. Immunophenotyping. Male. Middle Aged. Prognosis

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  • (PMID = 19821821.001).
  • [ISSN] 1365-2141
  • [Journal-full-title] British journal of haematology
  • [ISO-abbreviation] Br. J. Haematol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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28. Girón-González JA, Baturone R, Soto MJ, Márquez M, Macías I, Montes de Oca M, Medina F, Chozas N, García-Pérez S: Implications of immunomodulatory interleukins for the hyperimmunoglobulinemia of Sjögren's syndrome. Cell Immunol; 2009;259(1):56-60
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  • The levels of IL-4 were notably higher in pSS patients with monoclonal gammopathy.
  • In conclusion, patients with pSS show a state of macrophage and T-lymphocyte activation with increased concentrations of cytokines implicated in the differentiation of B cells and secretion of immunoglobulins.
  • [MeSH-major] Hypergammaglobulinemia / blood. Immunoglobulins / blood. Interleukins / blood. Sjogren's Syndrome / blood

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  • (PMID = 19540455.001).
  • [ISSN] 1090-2163
  • [Journal-full-title] Cellular immunology
  • [ISO-abbreviation] Cell. Immunol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Antinuclear; 0 / Immunoglobulins; 0 / Interleukins
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29. Jagannath S, Kyle RA, Palumbo A, Siegel DS, Cunningham S, Berenson J: The current status and future of multiple myeloma in the clinic. Clin Lymphoma Myeloma Leuk; 2010 Feb;10(1):28-43
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  • It is now recognized that all cases of multiple myeloma (MM) are preceded by the premalignant condition of monoclonal gammopathy of undetermined significance (MGUS).
  • Although patients with MGUS are generally asymptomatic and currently managed by "watch and wait," the identification of high-risk patients whose disease will progress more rapidly to smoldering MM (SMM) and MM aids in timely intervention.
  • This review highlights challenges in the clinic and newer approaches under evaluation for the treatment and/or management of patients with MGUS, SMM, and MM.
  • [MeSH-minor] Clinical Trials as Topic. Humans. Monoclonal Gammopathy of Undetermined Significance / drug therapy

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  • (PMID = 20223727.001).
  • [ISSN] 2152-2669
  • [Journal-full-title] Clinical lymphoma, myeloma & leukemia
  • [ISO-abbreviation] Clin Lymphoma Myeloma Leuk
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 148
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30. San Miguel JF, García-Sanz R: Prognostic features of multiple myeloma. Best Pract Res Clin Haematol; 2005;18(4):569-83
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  • Accordingly, investigation of prognostic factors may contribute to identification of risk categories and to provision of more accurate information about individual disease outcome.
  • Subsequently, biological parameters such as the proliferative activity of plasma cells and beta2-microglobulin have been added to the prognostic arsenal.
  • More recently, cytogenetic and molecular markers with significant influence on disease outcome have been identified.
  • Here we will review the most relevant prognostic factors reported in the literature in patients treated with both conventional chemotherapy and high-dose therapy followed by autologous stem-cell support, as well as in asymptomatic MM and MGUS patients.
  • [MeSH-major] Multiple Myeloma / diagnosis
  • [MeSH-minor] Hematopoietic Stem Cell Transplantation / methods. Humans. Prognosis. Risk Factors. Treatment Outcome. Tumor Burden

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  • (PMID = 16026738.001).
  • [ISSN] 1521-6926
  • [Journal-full-title] Best practice & research. Clinical haematology
  • [ISO-abbreviation] Best Pract Res Clin Haematol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 99
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31. Pangalis GA, Kyrtsonis MC, Kontopidou FN, Siakantaris MP, Dimopoulou MN, Vassilakopoulos TP, Tzenou T, Kokoris S, Dimitriadou E, Kalpadakis C, Tsalimalma K, Tsaftaridis P, Panayiotidis P, Angelopoulou MK: Differential diagnosis of Waldenstrom's macroglobulinemia and other B-cell disorders. Clin Lymphoma; 2005 Mar;5(4):235-40
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  • [Title] Differential diagnosis of Waldenstrom's macroglobulinemia and other B-cell disorders.
  • Waldenstrom's macroglobulinemia (WM) is characterized by lymphoplasmacytic infiltration of bone marrow and/or other tissues and by the presence of serum monoclonal immunoglobulin M ([IgM], without cutoff limit).
  • Differential diagnosis from other B-cell disorders (BCDs) is usually easy based on clinical, morphologic, histopathologic, immunophenotypic, and genetic features.
  • However, all BCDs potentially produce monoclonal IgM.
  • Eighty-four patients were diagnosed with WM, 5 with IgM-monoclonal gammopathy of undetermined significance (MGUS), and 41 with other BCDs (9 with B-cell chronic lymphocytic leukemia, 5 with small lymphocytic lymphoma, 14 with marginal zone lymphoma, 5 with mantle-cell lymphoma, 2 with follicular lymphoma, 2 with diffuse large B-cell lymphoma, 2 with cryoglobulinemia, and 2 with low-grade lymphoma not otherwise specified).
  • Median IgM levels were 3215 mg/dL in WM, 840 mg/dL in IgM-MGUS, and 285 mg/dL in other BCDs (5 had IgM levels > 1500 mg/dL).
  • In 10% of non-WM BCDs, monoclonal IgM was found only when more sensitive immunofixation methods were used.
  • Careful diagnosis requires the concomitant evaluation of all parameters of BCDs together.
  • Special attention should be given to mantle cell lymphoma in its atypical forms.
  • Research in this field should continue to further clarify the disease entities that overlap with WM.
  • [MeSH-major] Immunoglobulin M / analysis. Leukemia, Lymphocytic, Chronic, B-Cell / diagnosis. Lymphoma / diagnosis. Waldenstrom Macroglobulinemia / diagnosis
  • [MeSH-minor] Aged. Diagnosis, Differential. Female. Gene Expression Profiling. Humans. Lymphoma, Mantle-Cell / diagnosis. Lymphoma, Mantle-Cell / immunology. Male. Middle Aged. Retrospective Studies

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  • (PMID = 15794855.001).
  • [ISSN] 1526-9655
  • [Journal-full-title] Clinical lymphoma
  • [ISO-abbreviation] Clin Lymphoma
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulin M
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32. Zheng JM, Chen S, Zhang WQ, Pan XZ, Zhu HX, Jiang WM: [Misdiagnosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome: a case report]. Zhonghua Gan Zang Bing Za Zhi; 2007 Aug;15(8):626-7
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  • [Title] [Misdiagnosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome: a case report].
  • [MeSH-major] Diagnostic Errors. POEMS Syndrome / diagnosis
  • [MeSH-minor] Ascites / complications. Ascites / diagnosis. Humans. Liver Cirrhosis / complications. Liver Cirrhosis / diagnosis. Male. Middle Aged

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  • (PMID = 17711642.001).
  • [ISSN] 1007-3418
  • [Journal-full-title] Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology
  • [ISO-abbreviation] Zhonghua Gan Zang Bing Za Zhi
  • [Language] chi
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China
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33. van Rhee F: Light-chain MGUS: implications for clinical practice. Lancet; 2010 May 15;375(9727):1670-1
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  • [Title] Light-chain MGUS: implications for clinical practice.
  • [MeSH-major] Immunoglobulin Light Chains / blood. Monoclonal Gammopathy of Undetermined Significance / complications
  • [MeSH-minor] Aged. Aged, 80 and over. Humans. Middle Aged. Multiple Myeloma / etiology. Precancerous Conditions / complications. Precancerous Conditions / diagnosis. Precancerous Conditions / epidemiology. Risk Factors

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  • [CommentOn] Lancet. 2010 May 15;375(9727):1721-8 [20472173.001]
  • (PMID = 20472153.001).
  • [ISSN] 1474-547X
  • [Journal-full-title] Lancet (London, England)
  • [ISO-abbreviation] Lancet
  • [Language] eng
  • [Publication-type] Comment; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Immunoglobulin Light Chains
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34. Bergón E, Miravalles E, Bergón E, Miranda I, Bergón M: The predictive power of serum kappa/lambda ratios for discrimination between monoclonal gammopathy of undetermined significance and multiple myeloma. Clin Chem Lab Med; 2005;43(1):32-7
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  • [Title] The predictive power of serum kappa/lambda ratios for discrimination between monoclonal gammopathy of undetermined significance and multiple myeloma.
  • The predictive power of serum kappa/lambda ratios on initial presentation of immunoglobulin G (IgG) or IgA monoclonal component was studied to differentiate between monoclonal gammopathy of undetermined significance (MGUS) and multiple myeloma (MM) patients.
  • The retrospective study involved 145 patients clinically diagnosed with monoclonal gammopathy of undetermined significance or multiple myeloma, who had serum M-protein IgG <35 g/L or IgA <20 g/L at M-protein detection.
  • MM patients were considered as diseased and MGUS patients as non-diseased in order to estimate the performance characteristics of serum kappa/lambda ratios.
  • There was a statistically significant difference in kappa/lambda ratios distribution between both groups of patients, in both M-protein kappa-type (Mann-Whitney U=168, p<0.001) and in M-protein lambda-type (Mann-Whitney U=143, p<0.001).
  • Negative likelihood ratios at threshold levels of 0.6 and 4.2 were 2.17- and 3.32-fold greater, respectively, than positive likelihood ratios, so that the predictive power of a serum kappa/lambda ratio within these limits is better in ruling out (negative predictive power) than ruling in disease (positive predictive power).
  • The post-test characteristics of a serum kappa/lambda ratio interval between 0.6 and 4.2 in discriminating MGUS from MM in our geographic population were: sensitivity 0.96 (0.93-0.99 95% CI); specificity 0.70 (0.63-0.77); positive predictive value 0.68 (0.64-0.73); negative predictive value 0.96 (0.94-0.99); likelihood ratios (+)LR 3.23 (2.68-4.04); and (-)LR 17.16 (11.00-63.00).
  • Thus, serum M-protein with a kappa/lambda ratio between 0.6 and 4.2 increases the posterior probability of MGUS from 0.60 to 0.96 in asymptomatic patients, for whom only monitoring may be suggested when the serum kappa/lambda ratio is within these limits.

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  • [ErratumIn] Clin Chem Lab Med. 2005;43(3):349
  • (PMID = 15653439.001).
  • [ISSN] 1434-6621
  • [Journal-full-title] Clinical chemistry and laboratory medicine
  • [ISO-abbreviation] Clin. Chem. Lab. Med.
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Immunoglobulin G; 0 / Immunoglobulin M; 0 / Immunoglobulin kappa-Chains; 0 / Immunoglobulin lambda-Chains; 0 / Myeloma Proteins; 0 / multiple myeloma M-proteins
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35. Finsterer J, Stöllberger C, Avanzini M, Rauschka H: Restrictive cardiomyopathy in dermatomyositis. Scand J Rheumatol; 2006 May-Jun;35(3):229-32
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  • In a 78-year-old male with a history of arterial hypertension, recurrent episodes of atrial fibrillation and syncopes, rupture of an infra-renal aortic aneurysm with complications (recurrent QT-prolongation, lumbo-sacral plexopathy, transient ischaemic attack, peripheral embolism), monoclonal gammopathy, subdural haematoma, focal seizures, megaloblastic anaemia, leucopenia, eosinophilia, elevated muscle enzymes and increasing tiredness, dermatomyositis was diagnosed upon clinical presentation, muscle enzyme and muscle biopsy findings.
  • Patients with dermatomyositis should undergo a comprehensive cardiological investigation as soon as the neurological diagnosis is established.


36. Shin JW, Park R, Choi TY: [A case of multiple myeloma showing marked differences in serum IgG levels between protein electrophoresis and turbidimetry]. Korean J Lab Med; 2008 Aug;28(4):282-5
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  • Serum protein level was 14.4 g/dL at the time of diagnosis.
  • According to this, we considered that both protein electrophoresis and turbidimetry should be needed to quantify serum immunoglobulins for diagnosis and follow-up of the patients with monoclonal gammopathy.
  • [MeSH-major] Immunoglobulin G / blood. Multiple Myeloma / diagnosis

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  • (PMID = 18728377.001).
  • [ISSN] 1598-6535
  • [Journal-full-title] The Korean journal of laboratory medicine
  • [ISO-abbreviation] Korean J Lab Med
  • [Language] kor
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Korea (South)
  • [Chemical-registry-number] 0 / Immunoglobulin G; 0 / Immunoglobulin kappa-Chains
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37. Arai M, Kusunoki S: [A case of multifocal motor neuropathy with IgM lambda anti-GM1 antibody and IgM kappa paraprotein reacting exclusively with GM2]. Rinsho Shinkeigaku; 2009 Feb-Mar;49(2-3):123-6
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  • A diagnosis of multifocal motor neuropathy was made.
  • The patient had IgM kappa monoclonal gammopathy of undetermined significance.
  • [MeSH-major] G(M1) Ganglioside / immunology. G(M2) Ganglioside / immunology. Immunoglobulin M / immunology. Immunoglobulin kappa-Chains / immunology. Immunoglobulin lambda-Chains / immunology. Motor Neuron Disease / immunology. Paraproteins / immunology

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  • (PMID = 19348179.001).
  • [ISSN] 0009-918X
  • [Journal-full-title] Rinshō shinkeigaku = Clinical neurology
  • [ISO-abbreviation] Rinsho Shinkeigaku
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Immunoglobulin M; 0 / Immunoglobulin kappa-Chains; 0 / Immunoglobulin lambda-Chains; 0 / Paraproteins; 19600-01-2 / G(M2) Ganglioside; 37758-47-7 / G(M1) Ganglioside
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38. Rongioletti F: Lichen myxedematosus (papular mucinosis): new concepts and perspectives for an old disease. Semin Cutan Med Surg; 2006 Jun;25(2):100-4
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  • [Title] Lichen myxedematosus (papular mucinosis): new concepts and perspectives for an old disease.
  • A third group of atypical or intermediate forms, not meeting the criteria for either scleromyxedema or the localized form, includes cases of (1) scleromyxedema without monoclonal gammopathy, (2) localized forms with monoclonal gammopathy and/or systemic symptoms, (3) localized forms with mixed features of the subtypes, and (4) not well-specified cases.

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  • (PMID = 16908401.001).
  • [ISSN] 1085-5629
  • [Journal-full-title] Seminars in cutaneous medicine and surgery
  • [ISO-abbreviation] Semin Cutan Med Surg
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 21
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39. Mahapatra M, Mishra P, Makharia G, Kumar R, Saxena R: Acquired von Willebrand's disease associated with gastrointestinal angiodysplasia and monoclonal gammopathy. J Assoc Physicians India; 2006 Dec;54:963
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  • [Title] Acquired von Willebrand's disease associated with gastrointestinal angiodysplasia and monoclonal gammopathy.

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  • (PMID = 17334020.001).
  • [ISSN] 0004-5772
  • [Journal-full-title] The Journal of the Association of Physicians of India
  • [ISO-abbreviation] J Assoc Physicians India
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] India
  • [Chemical-registry-number] 0 / Immunoglobulins, Intravenous
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40. Wiedermann CJ, Kob M, Benvenuti S, Carella R, Lucchin L, Piazzi L, Chilovi F, Mazzoleni G: Digital clubbing in primary intestinal lymphangiectasia: a case report. Wien Med Wochenschr; 2010 Aug;160(15-16):431-6
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  • Primary intestinal lymphangiectasia (PIL), also known as Waldmann's disease, is a rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia.
  • We report a case of secondary hyperparathyroidism, osteopenia, monoclonal gammopathy and digital clubbing in a 57-year-old patient with a 12-year history of discontinuous diarrhea.
  • [MeSH-minor] Biopsy. Combined Modality Therapy. Diet, Fat-Restricted. Double-Balloon Enteroscopy. Enteral Nutrition. Food, Formulated. Humans. Intestine, Small / pathology. Lymphangiectasis, Intestinal / diagnosis. Lymphangiectasis, Intestinal / diet therapy. Lymphangiectasis, Intestinal / pathology. Lymphedema / diagnosis. Lymphedema / diet therapy. Lymphedema / pathology. Male. Middle Aged. Protein-Losing Enteropathies / diagnosis. Protein-Losing Enteropathies / diet therapy. Protein-Losing Enteropathies / pathology

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  • (PMID = 20812055.001).
  • [ISSN] 1563-258X
  • [Journal-full-title] Wiener medizinische Wochenschrift (1946)
  • [ISO-abbreviation] Wien Med Wochenschr
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Austria
  • [Chemical-registry-number] Waldmann disease
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41. Denguir H, Ben-Hamda K, Gharbi M, Addad F, Hammami S, Mlika A, Abbes F, Moussa A, Betbout F, Gamra H, Maatouk F, Dridi Z, Zakhama A, Mahjoub S, Ben-Farhat M: [Cardiac amyloidosis revealing multiple myeloma]. Tunis Med; 2005 May;83(5):300-4
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  • INTRODUCTION: Amyloidosis is a rare disease characterized by an extracellular accumulation of a protein polysaccharid complex (Amyloid).
  • Cardiac involvement is considered as a major prognostic factor.
  • The diagnosis of cardiac amyloidosis was suggested by echocardiography: Left ventricular concentric hypertrophy and typical amyeloid infiltration with hyperechoic, shiny and granite-like aspect of the interventricular septum.
  • This cardiac amyloidosis was secondary to multiple myeloma: monoclonal Gammopathy with immunoglobulin Lambda in the first and Kappa in the second, and the presence of a plasmocyte infiltration in the sternal puncture.
  • [MeSH-major] Amyloidosis / etiology. Heart Diseases / etiology. Multiple Myeloma / complications. Multiple Myeloma / diagnosis


42. Beyer M, Kochanek M, Giese T, Endl E, Weihrauch MR, Knolle PA, Classen S, Schultze JL: In vivo peripheral expansion of naive CD4+CD25high FoxP3+ regulatory T cells in patients with multiple myeloma. Blood; 2006 May 15;107(10):3940-9
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  • In healthy individuals, T(reg) cells consist not only of memory but also of naive T cells, which can undergo peripheral expansion and are characterized by a relative enrichment for autoreactive T-cell receptors.
  • Here, we demonstrate in patients with premalignant monoclonal gammopathy of undetermined significance and patients with multiple myeloma that functional FoxP3(+) T(reg) cells of naive, central, and effector memory phenotype as determined by CCR7 and CD45RA expression are significantly expanded.
  • Low frequencies of T-cell receptor excision circles in naive T(reg) cells in both healthy controls and multiple myeloma patients point to peripheral expansion as the prominent mechanism of increased frequencies of naive T(reg) cells in these cancer patients.
  • [MeSH-minor] Antigens, CD / blood. Antigens, CD / genetics. Antigens, CD45 / blood. Antigens, CD45 / genetics. Flow Cytometry. Humans. Receptors, Antigen, T-Cell / blood. Receptors, Antigen, T-Cell / immunology. Receptors, CCR7. Receptors, Chemokine / blood. Receptors, Chemokine / genetics

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  • (PMID = 16410445.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / CCR7 protein, human; 0 / FOXP3 protein, human; 0 / Forkhead Transcription Factors; 0 / Receptors, Antigen, T-Cell; 0 / Receptors, CCR7; 0 / Receptors, Chemokine; 0 / Receptors, Interleukin-2; EC 3.1.3.48 / Antigens, CD45
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43. Spisek R, Kukreja A, Chen LC, Matthews P, Mazumder A, Vesole D, Jagannath S, Zebroski HA, Simpson AJ, Ritter G, Durie B, Crowley J, Shaughnessy JD Jr, Scanlan MJ, Gure AO, Barlogie B, Dhodapkar MV: Frequent and specific immunity to the embryonal stem cell-associated antigen SOX2 in patients with monoclonal gammopathy. J Exp Med; 2007 Apr 16;204(4):831-40
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  • [Title] Frequent and specific immunity to the embryonal stem cell-associated antigen SOX2 in patients with monoclonal gammopathy.
  • Specific targets of cellular immunity in human premalignancy are largely unknown.
  • Monoclonal gammopathy of undetermined significance (MGUS) represents a precursor lesion to myeloma (MM).
  • We show that antigenic targets of spontaneous immunity in MGUS differ from MM.
  • MGUS patients frequently mount a humoral and cellular immune response against SOX2, a gene critical for self-renewal in embryonal stem cells.
  • Intranuclear expression of SOX2 marks the clonogenic CD138(-) compartment in MGUS.
  • Cellular immunity to SOX2 inhibits the clonogenic growth of MGUS cells in vitro.
  • Detection of anti-SOX2 T cells predicts favorable clinical outcome in patients with asymptomatic plasmaproliferative disorders.

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  • (PMID = 17389240.001).
  • [ISSN] 0022-1007
  • [Journal-full-title] The Journal of experimental medicine
  • [ISO-abbreviation] J. Exp. Med.
  • [Language] ENG
  • [Grant] United States / NCCIH NIH HHS / AT / P50 AT002779; United States / NCRR NIH HHS / RR / M01 RR000102; United States / NCI NIH HHS / CA / P01-CA55819; United States / NCI NIH HHS / CA / CA106802; United States / NCI NIH HHS / CA / CA109465; United States / NCI NIH HHS / CA / R01 CA106802; United States / NCCIH NIH HHS / AT / P50-AT02779; United States / NCI NIH HHS / CA / R01 CA109465; United States / NCI NIH HHS / CA / P01 CA055819; United States / NCRR NIH HHS / RR / M01-RR00102
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers; 0 / HMGB Proteins; 0 / Immunoglobulin G; 0 / SOX2 protein, human; 0 / SOXB1 Transcription Factors; 0 / Transcription Factors
  • [Other-IDs] NLM/ PMC2118551
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44. Kristinsson SY, Goldin LR, Björkholm M, Turesson I, Landgren O: Risk of solid tumors and myeloid hematological malignancies among first-degree relatives of patients with monoclonal gammopathy of undetermined significance. Haematologica; 2009 Aug;94(8):1179-81
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  • [Title] Risk of solid tumors and myeloid hematological malignancies among first-degree relatives of patients with monoclonal gammopathy of undetermined significance.

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  • [Cites] Blood. 2000 Mar 1;95(5):1869-71 [10688850.001]
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  • (PMID = 19546435.001).
  • [ISSN] 1592-8721
  • [Journal-full-title] Haematologica
  • [ISO-abbreviation] Haematologica
  • [Language] ENG
  • [Grant] United States / Intramural NIH HHS / /
  • [Publication-type] Letter; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't
  • [Publication-country] Italy
  • [Other-IDs] NLM/ PMC2719044
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45. de Seigneux S, Bindi P, Debiec H, Alyanakian MA, Aymard B, Callard P, Ronco P, Aucouturier P: Immunoglobulin deposition disease with a membranous pattern and a circulating monoclonal immunoglobulin G with charge-dependent aggregation properties. Am J Kidney Dis; 2010 Jul;56(1):117-21
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  • [Title] Immunoglobulin deposition disease with a membranous pattern and a circulating monoclonal immunoglobulin G with charge-dependent aggregation properties.
  • A 62-year-old woman presented with nephrotic syndrome, monoclonal gammopathy, and membranous-like nephropathy with nonorganized deposits composed of monoisotypic immunoglobulin G1 lambda protein.
  • Nephrotic syndrome remitted after a brief course of treatment with melphalan despite ongoing production of the monoclonal protein.
  • The circulating monoclonal immunoglobulin G1 lambda showed unusual in vitro aggregation properties, including dependence on low ionic strength and neutral pH, suggesting that electrostatic interactions had a role in the precipitation process.
  • This case illustrates the importance of looking for monoclonal immunoglobulin deposits when kidney biopsy findings are suggestive of membranous nephropathy.
  • [MeSH-major] Glomerulonephritis, Membranous / blood. Glomerulonephritis, Membranous / diagnosis. Immunoglobulin G / blood. Monoclonal Gammopathy of Undetermined Significance / blood. Monoclonal Gammopathy of Undetermined Significance / diagnosis
  • [MeSH-minor] Female. Humans. Middle Aged. Paraproteinemias / blood. Paraproteinemias / diagnosis

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  • (PMID = 20471736.001).
  • [ISSN] 1523-6838
  • [Journal-full-title] American journal of kidney diseases : the official journal of the National Kidney Foundation
  • [ISO-abbreviation] Am. J. Kidney Dis.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulin G
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46. Schuster C, Kränke B, Aberer E, Arbab E, Sturm G, Aberer W: Schnitzler syndrome: response to anakinra in two cases and a review of the literature. Int J Dermatol; 2009 Nov;48(11):1190-4
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  • BACKGROUND: Schnitzler syndrome is a rare disease characterized by a chronic urticarial eruption and monoclonal gammopathy, as well as clinical and laboratory signs of inflammation.
  • The pathophysiology is still unknown, although various autoantibody-mediated mechanisms have been described.
  • RESULTS: We report two cases of nearly complete remission of symptoms in Schnitzler syndrome after the initiation of anakinra therapy, and the first observation of a relapse under continuous daily anakinra therapy.

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  • (PMID = 20064173.001).
  • [ISSN] 1365-4632
  • [Journal-full-title] International journal of dermatology
  • [ISO-abbreviation] Int. J. Dermatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antirheumatic Agents; 0 / Interleukin 1 Receptor Antagonist Protein
  • [Number-of-references] 35
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47. Varella TC, Nishimura MY, Machado MC, de Moraes-Vasconcelos D, Rivitti EA: Schnitzler's syndrome without monoclonal gammopathy. Acta Derm Venereol; 2005;85(3):272-3
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  • [Title] Schnitzler's syndrome without monoclonal gammopathy.
  • [MeSH-major] Arthralgia / diagnosis. Immunoglobulin M
  • [MeSH-minor] Adult. Anti-Inflammatory Agents, Non-Steroidal / administration & dosage. Diagnosis, Differential. Drug Therapy, Combination. Humans. Male. Prednisone / administration & dosage. Syndrome. Thalidomide / administration & dosage. Urticaria / etiology. Urticaria / pathology

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  • (PMID = 16040423.001).
  • [ISSN] 0001-5555
  • [Journal-full-title] Acta dermato-venereologica
  • [ISO-abbreviation] Acta Derm. Venereol.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] Norway
  • [Chemical-registry-number] 0 / Anti-Inflammatory Agents, Non-Steroidal; 0 / Immunoglobulin M; 4Z8R6ORS6L / Thalidomide; VB0R961HZT / Prednisone
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48. Suurmeijer AJ: Papillary hemangiomas and glomeruloid hemangiomas are distinct clinicopathological entities. Int J Surg Pathol; 2010 Feb;18(1):48-54
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  • Thin basement membranes and glomeruloid architecture are typical of glomeruloid hemangiomas, whereas papillae with thick mantles of a basement membrane- like matrix enveloping pericytes are prominent in papillary hemangiomas.
  • This additional technique should allow pathologists to readily distinguish between the lesions and make a proper diagnosis.
  • What is important is that glomeruloid hemangiomas-often presenting in a spectrum of multiple cutaneous vascular lesions, including cherry hemangiomas-are a hallmark of POEMS (acronym for polyneuropathy, oganomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome and/or multicentric Castleman's disease, whereas papillary hemangiomas clinically present as innocent solitary cutaneous hemangiomas in otherwise healthy individuals.

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  • (PMID = 18805868.001).
  • [ISSN] 1940-2465
  • [Journal-full-title] International journal of surgical pathology
  • [ISO-abbreviation] Int. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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49. Brown LM, Gridley G, Check D, Landgren O: Risk of multiple myeloma and monoclonal gammopathy of undetermined significance among white and black male United States veterans with prior autoimmune, infectious, inflammatory, and allergic disorders. Blood; 2008 Apr 1;111(7):3388-94
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  • [Title] Risk of multiple myeloma and monoclonal gammopathy of undetermined significance among white and black male United States veterans with prior autoimmune, infectious, inflammatory, and allergic disorders.
  • In a retrospective cohort of more than 4 million white and black male United States (US) veterans, we explored the role of specific prior autoimmune, infectious, inflammatory, and allergic disorders in the etiology of multiple myeloma (MM) and monoclonal gammopathy of undetermined significance (MGUS).
  • The analysis included 4641 patients (3040 white, 1601 black) and 2046 patients (1312 white; 734 black) with a discharge diagnosis of MM and MGUS, respectively.
  • Using Poisson regression, we calculated age-adjusted relative risks (RRs) and 95% confidence intervals (CIs) for the relationship between MM, MGUS, and specific prior medical conditions.
  • Risks for MGUS were generally of similar magnitude.
  • Our results indicate that various types of immune-mediated conditions might act as triggers for MM/MGUS development.


50. Hamidou MA, Belizna C, Wiertlewsky S, Audrain M, Biron C, Grolleau JY, Mussini JM: Intravenous cyclophosphamide in refractory polyneuropathy associated with IgM monoclonal gammopathy: an uncontrolled open trial. Am J Med; 2005 Apr;118(4):426-30
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  • [Title] Intravenous cyclophosphamide in refractory polyneuropathy associated with IgM monoclonal gammopathy: an uncontrolled open trial.

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  • (PMID = 15808143.001).
  • [ISSN] 0002-9343
  • [Journal-full-title] The American journal of medicine
  • [ISO-abbreviation] Am. J. Med.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulin M; 8N3DW7272P / Cyclophosphamide
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56. Barde C, Masouyé I, Saurat JH, Le Gal FA: [Scleroedema adultorum Buschke in a diabetic subject: intravenous immunoglobulin therapy]. Ann Dermatol Venereol; 2009 Apr;136(4):360-3
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  • [Transliterated title] Scléroedème de Buschke chez un diabétique: traitement par immunoglobulines intraveineuses.
  • BACKGROUND: Scleroedema adultorum Buschke (SB) is a rare disease involving scleroedema of the neck and shoulders.
  • Three types of SB are distinguished: the first is acute and develops after an infectious disease, the second is of insidious evolution and is associated with monoclonal gammopathy, and the third is associated with type 2 diabetes.
  • PATIENTS AND METHODS: We report the case of a type 2 diabetic patient presenting with progressive, oedematous timbering of the trunk associated with impaired mobility, dysphagia and restrictive respiratory syndrome.
  • Treatment was mandatory because of the adverse impact of the disease.

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  • (PMID = 19361705.001).
  • [ISSN] 0151-9638
  • [Journal-full-title] Annales de dermatologie et de vénéréologie
  • [ISO-abbreviation] Ann Dermatol Venereol
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Immunoglobulins, Intravenous
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57. Amrani Hassani M, Filali Baba A, Alami M, Lahlou H: [Laboratory diagnostic and prognostic factors: Multiple myeloma in Morocco]. Sante; 2010 Oct-Dec;20(4):209-13
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  • ObjectivesThe purpose of this retrospective study was to clarify the factors for a laboratory diagnosis of multiple myeloma (Kahler's disease) and assess their prognostic value.MethodsThe study of all 10 patients with multiple myeloma seen during 2009 in the department of internal medicine and the haematology laboratory of HASSAN II University Hospital Centre in Fez.
  • The median concentration of the monoclonal component was 28 g/l.
  • Protein electrophoresis and myelography results are major diagnostic elements, because they make it possible to rule out a monoclonal gammopathy of undetermined significance, for which treatment differs completely from that for multiple myeloma.
  • [MeSH-major] Monoclonal Gammopathy of Undetermined Significance. Multiple Myeloma

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  • (PMID = 21320814.001).
  • [ISSN] 1157-5999
  • [Journal-full-title] Santé (Montrouge, France)
  • [ISO-abbreviation] Sante
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
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58. Kriangkum J, Taylor BJ, Reiman T, Belch AR, Pilarski LM: Origins of Waldenstrom's macroglobulinemia: does it arise from an unusual B-cell precursor? Clin Lymphoma; 2005 Mar;5(4):217-9
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  • [Title] Origins of Waldenstrom's macroglobulinemia: does it arise from an unusual B-cell precursor?
  • However, we found that the mutation of switch regions essential for CSR were present in IgM monoclonal gammopathy of unknown significance (MGUS) but absent from WM B cells, suggesting the possibility that not all IgM MGUS have the potential to give rise to WM, and further strengthening the hypothesis that the target cell in transformation to WM is an unusual type of B cell.
  • [MeSH-minor] Antigens, Bacterial. Cell Differentiation. Gene Rearrangement. Humans. Mutation. Paraproteinemias / immunology. Paraproteinemias / physiopathology

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  • (PMID = 15794851.001).
  • [ISSN] 1526-9655
  • [Journal-full-title] Clinical lymphoma
  • [ISO-abbreviation] Clin Lymphoma
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA 80963
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, Bacterial; 0 / Antigens, CD20; 0 / H antigen; 0 / Immunoglobulin M
  • [Number-of-references] 22
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59. Rajkumar SV: MGUS and smoldering multiple myeloma: update on pathogenesis, natural history, and management. Hematology Am Soc Hematol Educ Program; 2005;:340-5
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  • [Title] MGUS and smoldering multiple myeloma: update on pathogenesis, natural history, and management.
  • Monoclonal gammopathy of undetermined significance (MGUS) and smoldering multiple myeloma (SMM) are asymptomatic, pre-malignant disorders characterized by monoclonal plasma cell proliferation in the bone marrow and absence of end-organ damage.
  • Recent advances in the pathogenesis, natural history, and prognosis of MGUS and SMM are reviewed.
  • A new risk stratification system to determine the prognosis of MGUS using the size and type of M protein, and the serum free light chain assay is discussed.

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  • (PMID = 16304401.001).
  • [ISSN] 1520-4383
  • [Journal-full-title] Hematology. American Society of Hematology. Education Program
  • [ISO-abbreviation] Hematology Am Soc Hematol Educ Program
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA 107476; United States / NCI NIH HHS / CA / CA 62242
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Glycoproteins; 0 / Immunoglobulin Heavy Chains; 0 / Myeloma Proteins; 0 / multiple myeloma M-proteins; 0 / protein M (glycoprotein)
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60. Fish R, Pinney J, Jain P, Addison C, Jones C, Jayawardene S, Booth J, Howie AJ, Ghonemy T, Rajabali S, Roberts D, White L, Khan S, Morgan M, Cockwell P, Hutchison CA: The incidence of major hemorrhagic complications after renal biopsies in patients with monoclonal gammopathies. Clin J Am Soc Nephrol; 2010 Nov;5(11):1977-80
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  • [Title] The incidence of major hemorrhagic complications after renal biopsies in patients with monoclonal gammopathies.
  • BACKGROUND AND OBJECTIVES: Monoclonal gammopathies frequently cause renal disease, but they may be an incidental finding.
  • Assessment of renal pathology in the context of renal dysfunction and a monoclonal gammopathy therefore serves as a useful diagnostic tool and, in addition, provides prognostic information.
  • The purpose of this study was to determine the incidence of significant hemorrhagic complications after renal biopsies in patients with monoclonal gammopathies.
  • Subjects were categorized as having a monoclonal gammopathy or not, and the incidence of major hemorrhagic complications between groups was compared.
  • One hundred forty-eight subjects with a monoclonal gammopathy were identified.
  • CONCLUSIONS: In the population studied, the rate of major hemorrhagic complications after percutaneous renal biopsy was not significantly greater in patients with a monoclonal gammopathy.
  • [MeSH-major] Hemorrhage / etiology. Kidney / pathology. Kidney Diseases / diagnosis. Paraproteinemias / complications

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  • (PMID = 20651154.001).
  • [ISSN] 1555-905X
  • [Journal-full-title] Clinical journal of the American Society of Nephrology : CJASN
  • [ISO-abbreviation] Clin J Am Soc Nephrol
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC3001781
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61. Baughn LB, Di Liberto M, Niesvizky R, Cho HJ, Jayabalan D, Lane J, Liu F, Chen-Kiang S: CDK2 phosphorylation of Smad2 disrupts TGF-beta transcriptional regulation in resistant primary bone marrow myeloma cells. J Immunol; 2009 Feb 15;182(4):1810-7
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  • In multiple myeloma, a fatal cancer of plasma cells, malignant cells accumulate in the TGF-beta-rich bone marrow due to loss of both cell cycle and apoptotic controls.
  • Herein we show that TGF-beta activates Smad2 but fails to induce cell cycle arrest or apoptosis in primary bone marrow myeloma and human myeloma cell lines due to its inability to activate G(1) cyclin-dependent kinase (CDK) inhibitors (p15(INK4b), p21(CIP1/WAF1), p27(KIP1), p57(KIP2)) or to repress c-myc and Bcl-2 transcription.
  • Correlating with aberrant activation of CDKs, CDK-dependent phosphorylation of Smad2 on Thr(8) (pT8), a modification linked to impaired Smad activity, is elevated in primary bone marrow myeloma cells, even in asymptomatic monoclonal gammopathy of undetermined significance.
  • Our findings provide the first direct evidence that pT8 Smad2 couples dysregulation of CDK2 to TGF-beta resistance in primary cancer cells, and they suggest that disruption of Smad2 function by CDK2 phosphorylation acts as a mechanism for TGF-beta resistance in multiple myeloma.

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  • (PMID = 19201832.001).
  • [ISSN] 1550-6606
  • [Journal-full-title] Journal of immunology (Baltimore, Md. : 1950)
  • [ISO-abbreviation] J. Immunol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / R01 CA120531; United States / NCI NIH HHS / CA / R01 CA93771
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / SMAD2 protein, human; 0 / Smad2 Protein; 0 / Transforming Growth Factor beta; EC 2.7.11.22 / CDK2 protein, human; EC 2.7.11.22 / Cyclin-Dependent Kinase 2
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62. Ramchandren S, Lewis RA: Monoclonal gammopathy and neuropathy. Curr Opin Neurol; 2009 Oct;22(5):480-5
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  • [Title] Monoclonal gammopathy and neuropathy.
  • PURPOSE OF REVIEW: The management of peripheral neuropathy associated with monoclonal gammopathies has been advanced by recent clinical studies.
  • We review the causal association between monoclonal gammopathy and neuropathy, and critically review the recent evidence on treatment.
  • RECENT FINDINGS: IgM monoclonal gammopathy of undetermined significance (MGUS) is the most commonly found monoclonal gammopathy associated with neuropathy.
  • Neuropathies associated with specific lymphoproliferative disorders may not respond to treatments aimed at that disorder.
  • Standard immunomodulatory agents including steroids, intravenous immunoglobulin, and plasmapheresis have shown limited efficacy in IgM monoclonal gammopathy of undetermined significance.
  • Newer studies have shown promising results with rituximab, a monoclonal antibody which targets the B cell surface antigen CD20 and results in a rapid and sustained depletion of B cells.
  • SUMMARY: There is a clear association between peripheral neuropathy and IgM MGUS with characteristic clinical, electrophysiology and pathologic features that make the disorder distinct from chronic inflammatory demyelinating polyneuropathy.
  • The IgG and IgA monoclonal gammopathies are rarely associated with specific neuropathies.
  • Long-term studies looking at the association between specific immunologic markers and disease recurrence are needed to ultimately develop targeted therapies.

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  • (PMID = 19625962.001).
  • [ISSN] 1473-6551
  • [Journal-full-title] Current opinion in neurology
  • [ISO-abbreviation] Curr. Opin. Neurol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 57
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63. Kawagashira Y, Kondo N, Atsuta N, Iijima M, Koike H, Katsuno M, Tanaka F, Kusunoki S, Sobue G: IgM MGUS anti-MAG neuropathy with predominant muscle weakness and extensive muscle atrophy. Muscle Nerve; 2010 Sep;42(3):433-5
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  • [Title] IgM MGUS anti-MAG neuropathy with predominant muscle weakness and extensive muscle atrophy.
  • We report a patient with anti-myelin-associated glycoprotein (MAG) neuropathy, predominantly exhibiting severe motor symptoms, accompanied by extensive muscle atrophy mimicking Charcot-Marie-Tooth disease.
  • [MeSH-minor] Charcot-Marie-Tooth Disease / pathology. Diagnosis, Differential. Electrodiagnosis. Humans. Immunoglobulins, Intravenous / therapeutic use. Lower Extremity / pathology. Male. Middle Aged. Muscular Atrophy. Neural Conduction / physiology

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  • (PMID = 20665518.001).
  • [ISSN] 1097-4598
  • [Journal-full-title] Muscle & nerve
  • [ISO-abbreviation] Muscle Nerve
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulin M; 0 / Immunoglobulins, Intravenous; 0 / Myelin-Associated Glycoprotein
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64. Choi JH, Jo YJ, Gong SJ, Hong BW, Lee HJ, Son BK, Jun DW, Kim SH, Park YS, Seok JW: Unicentric castleman disease is not clearly distinguished from multicentric type: a case report. Clin Lymphoma Myeloma; 2008 Aug;8(4):256-9
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  • [Title] Unicentric castleman disease is not clearly distinguished from multicentric type: a case report.
  • Castleman disease (CD) was recently classified as a unicentric hyaline vascular variant, unicentric plasma cell variant, and multicentric plasma cell variant.
  • The patient experienced anemia, weight loss, elevated C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome), and plasmacytosis in bone marrow without human herpesvirus-8 or HIV.
  • After exploratory laparotomy and lymphadenectomy under presumptive diagnosis of CD, the patient's symptoms recovered, and CRP and ESR decreased.
  • Therefore, we suggest that unicentric CD is not clearly distinguished from multicentric, the type in this report, focusing on the useful role of CRP, ESR, and positron emission tomography/computed tomography in the disease activity of CD.
  • [MeSH-major] Giant Lymph Node Hyperplasia / diagnosis. Giant Lymph Node Hyperplasia / pathology

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  • (PMID = 18765316.001).
  • [ISSN] 1557-9190
  • [Journal-full-title] Clinical lymphoma & myeloma
  • [ISO-abbreviation] Clin Lymphoma Myeloma
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 9007-41-4 / C-Reactive Protein
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65. Hanamura I, Stewart JP, Huang Y, Zhan F, Santra M, Sawyer JR, Hollmig K, Zangarri M, Pineda-Roman M, van Rhee F, Cavallo F, Burington B, Crowley J, Tricot G, Barlogie B, Shaughnessy JD Jr: Frequent gain of chromosome band 1q21 in plasma-cell dyscrasias detected by fluorescence in situ hybridization: incidence increases from MGUS to relapsed myeloma and is related to prognosis and disease progression following tandem stem-cell transplantation. Blood; 2006 Sep 1;108(5):1724-32
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  • [Title] Frequent gain of chromosome band 1q21 in plasma-cell dyscrasias detected by fluorescence in situ hybridization: incidence increases from MGUS to relapsed myeloma and is related to prognosis and disease progression following tandem stem-cell transplantation.
  • Using fluorescence in situ hybridization we investigated amplification of chromosome band 1q21 (Amp1q21) in more than 500 untreated patients with monoclonal gammopathy of undetermined significance (MGUS; n = 14), smoldering multiple myeloma (SMM; n = 31), and newly diagnosed MM (n = 479) as well as 45 with relapsed MM.
  • The frequency of Amp1q21 was 0% in MGUS, 45% in SMM, 43% in newly diagnosed MM, and 72% in relapsed MM (newly diagnosed versus relapsed MM, P < .001).
  • Amp1q21 was detected in 10 of 12 patients whose disease evolved to active MM compared with 4 of 19 who remained with SMM (P < .001).
  • Patients with newly diagnosed MM with Amp1q21 had inferior 5-year event-free/overall survival compared with those lacking Amp1q21 (38%/52% versus 62%/78%, both P < .001).
  • The proportion of cells with Amp1q21 and the copy number of 1q21 tended to increase at relapse compared with diagnosis.
  • Our data suggest that Amp1q21 is associated with both disease progression and poor prognosis.

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  • (PMID = 16705089.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA55819; United States / NCI NIH HHS / CA / CA97513
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC1895503
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66. Hoffman-Snyder C, Smith BE: Neuromuscular disorders associated with paraproteinemia. Phys Med Rehabil Clin N Am; 2008 Feb;19(1):61-79, vi
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  • Neuromuscular disorders associated with monoclonal gammopathies are usually uncovered in approximately 10% of patients presenting with peripheral neuropathy complaints.
  • This discovery should prompt further evaluation for underlying plasma cell dyscrasias.
  • The most frequent monoclonal disorders associated with neuropathy are smoldering myeloma, multiple myeloma, Waldenström macroglobulinemia, solitary plasmacytoma, systemic immunoglobulin light chain (AL) amyloidosis, POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes), and cryoglobulinemia.
  • If these are excluded by careful evaluation the patient is classified as having monoclonal gammopathy of undetermined significance.

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  • (PMID = 18194750.001).
  • [ISSN] 1047-9651
  • [Journal-full-title] Physical medicine and rehabilitation clinics of North America
  • [ISO-abbreviation] Phys Med Rehabil Clin N Am
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 40
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67. Ashawesh K, Fiad TM: Spontaneous recovery of adrenal insufficiency in POEMS syndrome. Medscape J Med; 2009;11(1):21
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  • POEMS syndrome is a rare multisystem disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes.
  • We report the first case of spontaneous recovery of adrenal insufficiency in a patient with POEMS syndrome treated with autologous hematopoietic cell transplantation.
  • [MeSH-major] Adrenal Insufficiency / diagnosis. Adrenal Insufficiency / surgery. POEMS Syndrome / diagnosis. POEMS Syndrome / surgery
  • [MeSH-minor] Adult. Female. Hematopoietic Stem Cell Transplantation / methods. Humans. Remission Induction / methods. Treatment Outcome

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  • (PMID = 19295942.001).
  • [ISSN] 1934-1997
  • [Journal-full-title] Medscape journal of medicine
  • [ISO-abbreviation] Medscape J Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC2654695
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68. Imai N, Kitamura E, Tachibana T, Konishi T, Suzuki Y, Serizawa M, Okabe T, Fujita H: Efficacy of autologous peripheral blood stem cell transplantation in POEMS syndrome with polyneuropathy. Intern Med; 2007;46(3):135-8
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  • [Title] Efficacy of autologous peripheral blood stem cell transplantation in POEMS syndrome with polyneuropathy.
  • POEMS syndrome (POEMS) is a rare multi-system disease which is typically associated with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes.
  • We report herein a case of POEMS with progressive polyneuropathy resistant to high-dose intravenous immunoglobulin therapy who experienced improvement in polyneuropathy after the administration of high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation.
  • Improvements in polyneuropathy remain despite recognition of monoclonal gammopathy by immunofixation as of 1 year after treatment.
  • We demonstrated that autologous peripheral blood stem cell transplantation can improve clinical symptoms and signs due to polyneuropathy in POEMS even without achieving complete remission.
  • [MeSH-major] POEMS Syndrome / therapy. Peripheral Blood Stem Cell Transplantation

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  • (PMID = 17268130.001).
  • [ISSN] 1349-7235
  • [Journal-full-title] Internal medicine (Tokyo, Japan)
  • [ISO-abbreviation] Intern. Med.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Immunosuppressive Agents; 8N3DW7272P / Cyclophosphamide
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69. Vital A, Nedelec-Ciceri C, Vital C: Presence of crystalline inclusions in the peripheral nerve of a patient with IgA lambda monoclonal gammopathy of undetermined significance. Neuropathology; 2008 Oct;28(5):526-31
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  • [Title] Presence of crystalline inclusions in the peripheral nerve of a patient with IgA lambda monoclonal gammopathy of undetermined significance.
  • Association of a peripheral neuropathy with an IgA monoclonal gammopathy of undetermined significance (MGUS) is not commonly observed and is sometimes considered as coincidental.
  • We present a case in which the nerve biopsy revealed the presence of crystalline inclusions in the endoneurium, a very unusual finding.
  • An IgA lambda MGUS was evidenced in his serum at 10.2 g/L with 7% plasma cells in his bone marrow and no lytic lesion at skeletal examination.
  • Such crystalline inclusions have mainly been observed in the cytoplasm of plasma cells in cases of multiple myeloma, and correspond to non-secreted IgA or IgG immunoglobulins with a kappa or rarely lambda light chain.
  • Such inclusions have also been reported in the cytoplasm of the epithelial cells from corneal fragments, in patients with multiple myeloma or IgG MGUS, and in the tubular cells from the kidney of patients with multiple myeloma and a nephrotic syndrome.

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  • (PMID = 18410274.001).
  • [ISSN] 1440-1789
  • [Journal-full-title] Neuropathology : official journal of the Japanese Society of Neuropathology
  • [ISO-abbreviation] Neuropathology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Immunoglobulin A; 0 / Immunoglobulin lambda-Chains
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70. Blotta S, Tassone P, Prabhala RH, Tagliaferri P, Cervi D, Amin S, Jakubikova J, Tai YT, Podar K, Mitsiades CS, Zullo A, Franco B, Anderson KC, Munshi NC: Identification of novel antigens with induced immune response in monoclonal gammopathy of undetermined significance. Blood; 2009 Oct 08;114(15):3276-84
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  • [Title] Identification of novel antigens with induced immune response in monoclonal gammopathy of undetermined significance.
  • The transformation from monoclonal gammopathy of undetermined significance (MGUS) to multiple myeloma (MM) is thought to be associated with changes in immune processes.
  • We have therefore used serologic analysis of recombinant cDNA expression library to screen the sera of MGUS patients to identify tumor-associated antigens.
  • A total of 10 antigens were identified, with specific antibody responses in MGUS.
  • Importantly, the gene responsible for the oral-facial-digital type I syndrome (OFD1) had response in 6 of 29 (20.6%) MGUS patients but 0 of 11 newly diagnosed MM patients.
  • Interestingly, 3 of 11 (27.2%) MM patients after autologous stem cell transplantations showed responses to OFD1.
  • We have confirmed T-cell responses against OFD1 in MGUS and observed down-regulation of GLI1/PTCH1 and p-beta-catenin after OFD1 knock-down with specific siRNA, suggesting its functional role in the regulation of Hh and Wnt pathways.
  • These findings demonstrate OFD1 as an important immune target and highlight its possible role in signal transduction and tumorigenesis in MGUS and MM.
  • [MeSH-minor] Aged. Aged, 80 and over. Apoptosis Regulatory Proteins / genetics. Apoptosis Regulatory Proteins / immunology. Carrier Proteins / genetics. Carrier Proteins / immunology. DNA, Complementary / genetics. Female. Humans. Male. Nuclear Proteins / genetics. Nuclear Proteins / immunology. Patched Receptors. Patched-1 Receptor. RNA-Binding Proteins / genetics. RNA-Binding Proteins / immunology. Receptors, Cell Surface / genetics. Receptors, Cell Surface / immunology. Signal Transduction / immunology. Stem Cell Transplantation. T-Lymphocytes / immunology. Transplantation, Autologous. beta Catenin / genetics. beta Catenin / immunology

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  • (PMID = 19587378.001).
  • [ISSN] 1528-0020
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Grant] United States / PHS HHS / / P01-78378; Italy / Telethon / / TGM06Z08; United States / NCI NIH HHS / CA / P50CA-100707; United States / NCI NIH HHS / CA / P01 CA078378; United States / PHS HHS / / R01-129494; United States / NCI NIH HHS / CA / P01 CA155258; United States / NCI NIH HHS / CA / P50 CA100707
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Apoptosis Regulatory Proteins; 0 / Autoantigens; 0 / Carrier Proteins; 0 / DNA, Complementary; 0 / IRF2BP2 protein, human; 0 / Nuclear Proteins; 0 / OFD1 protein, human; 0 / PTCH protein, human; 0 / Patched Receptors; 0 / Patched-1 Receptor; 0 / Proteins; 0 / RNA-Binding Proteins; 0 / Receptors, Cell Surface; 0 / beta Catenin
  • [Other-IDs] NLM/ PMC2759650
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71. Schröder R, Deckert M, Linke RP: Novel isolated cerebral ALlambda amyloid angiopathy with widespread subcortical distribution and leukoencephalopathy due to atypical monoclonal plasma cell proliferation, and terminal systemic gammopathy. J Neuropathol Exp Neurol; 2009 Mar;68(3):286-99
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  • [Title] Novel isolated cerebral ALlambda amyloid angiopathy with widespread subcortical distribution and leukoencephalopathy due to atypical monoclonal plasma cell proliferation, and terminal systemic gammopathy.
  • Neuropathologic and biochemical findings in a 34-year-old man whose disease began 2 years before death appearing as chronic progressive encephalitis and culminated in mutism are reported.
  • Cerebrospinal fluid and serum of the patient showed a brain-restricted monoclonal lambda-light chain apparently produced by a small monoclonal immunoglobulin Glambda plasma cell population.
  • In the preterminal stage, there was a systemic monoclonal gammopathy, the source of which could not be identified.
  • At autopsy, there was extensive amyloid deposition in most vessels throughout the cerebral and cerebellar white matter, basal ganglia, and thalamus and diffuse leukoencephalopathy; cerebral and cerebellar cortices, other portions of the CNS, and non-CNS tissues were spared.
  • Partial amino acid sequence analysis demonstrated that the amyloidogenic protein originated from immunoglobulin lambda-light chains which were produced by monoclonal plasma cells.
  • [MeSH-major] Amyloid / metabolism. Cerebral Amyloid Angiopathy / pathology. Dementia, Vascular / pathology. Paraproteinemias / pathology. Plasma Cells / pathology

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  • (PMID = 19225408.001).
  • [ISSN] 0022-3069
  • [Journal-full-title] Journal of neuropathology and experimental neurology
  • [ISO-abbreviation] J. Neuropathol. Exp. Neurol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Amyloid; 0 / Immunoglobulin lambda-Chains; 0 / amyloid protein AL
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72. Tsai DE, Aqui NA, Tomaszewski JE, Olthoff KM, Ahya VN, Kotloff RM, Bloom RD, Brozena SC, Hodinka RL, Stadtmauer EA, Schuster SJ, Nasta SD, Porter DL, Luger SM, Klumpp TR: Serum protein electrophoresis abnormalities in adult solid organ transplant patients with post-transplant lymphoproliferative disorder. Clin Transplant; 2005 Oct;19(5):644-52
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  • [Title] Serum protein electrophoresis abnormalities in adult solid organ transplant patients with post-transplant lymphoproliferative disorder.
  • Post-transplant lymphoproliferative disorder (PTLD) is an Epstein-Barr virus (EBV) associated malignancy that occurs in the setting of pharmacologic immunosuppression used after organ transplantation.
  • The presence of monoclonal gammopathy (MG) after organ transplantation is a risk factor for the development of PTLD.
  • Background gammaglobulin levels were abnormal in 13 (34%) patients with five (13%) and eight (21%) having polyclonal hypergammaglobulinemia or hypogammaglobulinemia, respectively.
  • Hypogammaglobulinemia was correlated with the presence of MG (p = 0.01) and polymorphic B-cell hyperplasia histology (p = 0.01).

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  • (PMID = 16146557.001).
  • [ISSN] 0902-0063
  • [Journal-full-title] Clinical transplantation
  • [ISO-abbreviation] Clin Transplant
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Biomarkers; 0 / Blood Proteins; 0 / Immunosuppressive Agents
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73. Gregorek H, Chrzanowska KH, Dzierzanowska-Fangrat K, Wakulińska A, Pietrucha B, Zapaśnik A, Zborowska M, Pac M, Smółka-Afifi D, Kasztelewicz B, Piekutowska-Abramczuk D, Małdyk J: Nijmegen breakage syndrome: Long-term monitoring of viral and immunological biomarkers in peripheral blood before development of malignancy. Clin Immunol; 2010 Jun;135(3):440-7
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  • Selected viruses and immune parameters were monitored in 57 patients with Nijmegen breakage syndrome as a proposed tool for early detection of changes preceding development of malignancy.
  • (2) monoclonal proteins; and (3) B-cell and T-cell receptor gene rearrangements in peripheral blood lymphocytes.
  • Monoclonal gammopathy detected in 38.6% of cases correlated with the presence of EBV DNA (p=0.002) and HCV RNA (p=0.04).
  • Systematic PCR analysis for viral infections and Ig/TCR gene rearrangements, supplemented by detection of monoclonal proteins, is advantageous in monitoring NBS patients before severe complications of the disease, including cancer, appear.
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Female. Gene Rearrangement, B-Lymphocyte. Gene Rearrangement, T-Lymphocyte. Humans. Infant. Male. Paraproteinemias. Receptors, Antigen, B-Cell / genetics. Receptors, Antigen, T-Cell / genetics. Reverse Transcriptase Polymerase Chain Reaction. T-Lymphocyte Subsets / immunology. Young Adult

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  • [Copyright] Copyright 2010 Elsevier Inc. All rights reserved.
  • (PMID = 20167538.001).
  • [ISSN] 1521-7035
  • [Journal-full-title] Clinical immunology (Orlando, Fla.)
  • [ISO-abbreviation] Clin. Immunol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Receptors, Antigen, B-Cell; 0 / Receptors, Antigen, T-Cell
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74. Corre J, Mahtouk K, Attal M, Gadelorge M, Huynh A, Fleury-Cappellesso S, Danho C, Laharrague P, Klein B, Rème T, Bourin P: Bone marrow mesenchymal stem cells are abnormal in multiple myeloma. Leukemia; 2007 May;21(5):1079-88
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  • To address this hypothesis in multiple myeloma (MM), we studied bone marrow mesenchymal stem cells (BMMSCs), the only long-lived cells of the bone marrow microenvironment, by gene expression profiling and phenotypic and functional studies in three groups of individuals: patients with MM, patients with monoclonal gamopathy of undefined significance (MGUS) and healthy age-matched subjects.
  • MGUS BMMSCs were interspersed between these two groups.
  • In particular, GDF15 was found to induce dose-dependent growth of MOLP-6, a stromal cell-dependent myeloma cell line.
  • [MeSH-minor] Adult. Aged. Cell Differentiation. Cell Proliferation. Cytokines / genetics. Female. Gene Expression Profiling. Growth Differentiation Factor 15. Hematopoiesis. Humans. Male. Middle Aged. Osteoblasts / cytology

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  • (PMID = 17344918.001).
  • [ISSN] 0887-6924
  • [Journal-full-title] Leukemia
  • [ISO-abbreviation] Leukemia
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Cytokines; 0 / GDF15 protein, human; 0 / Growth Differentiation Factor 15
  • [Other-IDs] NLM/ HALMS270565; NLM/ PMC2346535
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75. Nobile-Orazio E: Update on neuropathies associated with monoclonal gammopathy of undetermined significance (2008-2010). J Peripher Nerv Syst; 2010 Dec;15(4):302-6
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  • [Title] Update on neuropathies associated with monoclonal gammopathy of undetermined significance (2008-2010).
  • Studies on paraproteinemic neuropathies have appeared in the last 2 years improving the diagnosis of these neuropathies, clarifying their pathogenesis, and informing practice by randomized clinical trial publications.
  • [MeSH-major] Paraproteinemias / diagnosis. Paraproteinemias / therapy. Polyneuropathies / diagnosis. Polyneuropathies / therapy
  • [MeSH-minor] Animals. Antibodies, Monoclonal, Murine-Derived / therapeutic use. Humans. Randomized Controlled Trials as Topic / methods. Rituximab

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  • [Copyright] © 2010 Peripheral Nerve Society.
  • (PMID = 21199101.001).
  • [ISSN] 1529-8027
  • [Journal-full-title] Journal of the peripheral nervous system : JPNS
  • [ISO-abbreviation] J. Peripher. Nerv. Syst.
  • [Language] eng
  • [Publication-type] Congresses
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal, Murine-Derived; 4F4X42SYQ6 / Rituximab
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76. Flórez AF, Gallardo Agromayor E, García-Barredo R, Landeras Alvaro RM, Gorriño Angulo M, Martínez-Taboada VM, Fernández Echevarría MA: Radiological aid to clinical diagnosis of Schnitzler's syndrome: multimodality imaging approach. Clin Rheumatol; 2008 Jan;27(1):107-10
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  • [Title] Radiological aid to clinical diagnosis of Schnitzler's syndrome: multimodality imaging approach.
  • Schnitzler's syndrome is a rare combination of chronic urticaria, fever of unknown origin, disabling bone pain, and monoclonal gammopathy.
  • We report a case with an unusual radiological manifestation as a solitary sclerotic lesion of the right iliac bone.

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  • (PMID = 17982708.001).
  • [ISSN] 0770-3198
  • [Journal-full-title] Clinical rheumatology
  • [ISO-abbreviation] Clin. Rheumatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
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77. Treon SP, Hunter ZR, Aggarwal A, Ewen EP, Masota S, Lee C, Santos DD, Hatjiharissi E, Xu L, Leleu X, Tournilhac O, Patterson CJ, Manning R, Branagan AR, Morton CC: Characterization of familial Waldenstrom's macroglobulinemia. Ann Oncol; 2006 Mar;17(3):488-94
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  • BACKGROUND: Familial clustering of B-cell disorders among Waldenström's macroglobulinemia (WM) patients has been reported, though the frequency and any differences in disease manifestation for familial patients remain to be defined.
  • Forty-eight (18.7%) patients had at least one first-degree relative with either WM (n = 13, 5.1%), or another B-cell disorder including non-Hodgkin's lymphoma (n = 9, 3.5%), myeloma (n = 8, 3.1%), chronic lymphocytic leukemia (n = 7, 2.7%), monoclonal gammopathy of unknown significance (n = 5, 1.9%), acute lymphocytic leukemia (n = 3, 1.2%) and Hodgkin's disease (n = 3, 1.2%).
  • Patients with a familial history of WM or a plasma cell disorder (PCD) were diagnosed at a younger age and with greater bone marrow involvement.
  • RESULTS: Deletions in 6q represented the only recurrent structural chromosomal abnormality and were found in 13% of patients, all non-familial cases.
  • CONCLUSIONS: The above results suggest a high degree of clustering for B-cell disorders among first-degree relatives of patients with WM, along with distinct clinical features at presentation based on familial disease cluster patterns.

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  • (PMID = 16357024.001).
  • [ISSN] 0923-7534
  • [Journal-full-title] Annals of oncology : official journal of the European Society for Medical Oncology
  • [ISO-abbreviation] Ann. Oncol.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / K23 CA087977-03; United States / NCI NIH HHS / CA / P30 CA06516
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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78. Joint Task Force of the EFNS and the PNS: European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of paraproteinemic demyelinating neuropathies. Report of a Joint Task Force of the European Federation of Neurological Societies and the Peripheral Nerve Society--first revision. J Peripher Nerv Syst; 2010 Sep;15(3):185-95
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  • [Title] European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of paraproteinemic demyelinating neuropathies. Report of a Joint Task Force of the European Federation of Neurological Societies and the Peripheral Nerve Society--first revision.
  • (1) patients with PDN should be investigated for a malignant plasma cell dyscrasia;.
  • (2) a monoclonal gammopathy of undetermined significance is more likely to be causing the neuropathy if it is immunoglobulin (Ig)M, anti-neural antibodies are present, and the clinical phenotype is chronic distal sensory neuropathy;.
  • Their potential benefit should be balanced against possible side effects and the usually slow disease progression;.
  • (5) IgG and IgA PDN may be indistinguishable from chronic inflammatory demyelinating polyradiculoneuropathy; and (6) Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes syndrome is a multi-system malignant PDN.
  • [MeSH-major] Paraproteinemias / diagnosis. Paraproteinemias / therapy. Polyradiculoneuropathy / diagnosis. Polyradiculoneuropathy / therapy

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  • [Copyright] © 2010 Peripheral Nerve Society.
  • (PMID = 21040140.001).
  • [ISSN] 1529-8027
  • [Journal-full-title] Journal of the peripheral nervous system : JPNS
  • [ISO-abbreviation] J. Peripher. Nerv. Syst.
  • [Language] eng
  • [Publication-type] Guideline; Journal Article
  • [Publication-country] United States
  • [Investigator] Hadden RD; Nobile-Orazio E; Sommer CL; Hahn AF; Illa I; Morra E; Pollard JD; Lunn MP; Bouche P; Cornblath DR; Evers E; Koski CL; Léger JM; Van den Bergh P; van Doorn PA; van Schaik IN
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79. Scudla V, Adam Z: [Diagnostic importance and pitfalls of evaluating bone marrow smear preparation in multiple myeloma]. Vnitr Lek; 2006 Nov;52 Suppl 2:55-65
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  • Special attention is given to the indication and technique of aspiration biopsy, diagnostic and differential-diagnostic importance of evaluating the percentage representation of plasmatic cells in the bone marrow and their morphological deviation from the point of view of distinguishing them from reactive plasmocytosis and monoclonal gammopathy of undetermined significance.
  • Also addressed is the issue of selected morphological classification systems including their benefits in identifying and forecasting the prognosis for the disease.
  • A large part of the report is dedicated to the interpretational limitations of evaluating BM smear samples for the diagnosis and monitoring of the course of MM, and due attention is also given to indication for and assessment of the importance of histological examination of a BM trepanobioptic sample of bone marrow and the benefits of particular specialized methods, especially the examination of immunophenotypic, proliferative and apoptotic qualities of plasmocytes.
  • [MeSH-minor] Biopsy, Needle. Bone Marrow Examination. Cytodiagnosis. Diagnosis, Differential. Humans

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  • (PMID = 18175430.001).
  • [ISSN] 0042-773X
  • [Journal-full-title] Vnitr̆ní lékar̆ství
  • [ISO-abbreviation] Vnitr Lek
  • [Language] cze
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Czech Republic
  • [Number-of-references] 38
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80. Spier BJ, Einstein M, Johnson EA, Zuricik AO 3rd, Hu JL, Pfau PR: Amyloidosis presenting as lower gastrointestinal hemorrhage. WMJ; 2008 Feb;107(1):40-3
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  • AL-Amyloid rarely presents in the gastrointestinal tract as acute gastrointestinal hemorrhage, especially in the absence of clinical disease elsewhere in the body.
  • There are no reported cases of monoclonal gammopathy of undetermined significance progressing to AL-Amyloid presenting as lower gastrointestinal hemorrhage.
  • We report a case of a patient initially diagnosed with monoclonal gammopathy of undetermined significance who progressed to AL-Amyloid over the course of 1 year.
  • The diagnosis was made by biopsy of a sigmoid plaque demonstrating necrotic material on histopathology.
  • We conclude that early endoscopic examination and biopsy of the surrounding intestinal tissue is indicated when patients with monoclonal gammopathy of undetermined significance present with gastrointestinal hemorrhage to evaluate for the progression to AL-Amyloidosis.
  • Treatment to prevent recurrent hemorrhage and further progression of the disease should be considered.
  • [MeSH-major] Amyloidosis / complications. Amyloidosis / diagnosis. Gastrointestinal Hemorrhage / etiology
  • [MeSH-minor] Aged. Diagnosis, Differential. Humans. Male


81. Kyle RA, Rajkumar SV, Therneau TM, Larson DR, Plevak MF, Melton LJ 3rd: Prognostic factors and predictors of outcome of immunoglobulin M monoclonal gammopathy of undetermined significance. Clin Lymphoma; 2005 Mar;5(4):257-60
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  • [Title] Prognostic factors and predictors of outcome of immunoglobulin M monoclonal gammopathy of undetermined significance.
  • Monoclonal gammopathy of undetermined significance (MGUS) of the immunoglobulin M (IgM) class was diagnosed at our institution in 213 patients who resided in the 11 counties of southeastern Minnesota from 1960 to 1994.
  • The median age at diagnosis was 74 years and the median concentration of serum M-protein was 1.2 g/dL.
  • During follow-up, non-Hodgkin's lymphoma (n = 17), Waldenstrom's macroglobulinemia (n = 6), primary amyloidosis (n = 3), and chronic lymphocytic leukemia (n = 3) developed in 29 patients (14%).
  • Multivariate analysis revealed that only the concentration of serum M-protein at diagnosis and the serum albumin value were independent predictors of progression.
  • It was concluded that the patients with IgM MGUS should be followed indefinitely.
  • [MeSH-minor] Adult. Age of Onset. Aged. Aged, 80 and over. Disease Progression. Female. Follow-Up Studies. Humans. Lymphoma / pathology. Male. Middle Aged. Multivariate Analysis. Prognosis. Risk Factors. Survival Analysis

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  • (PMID = 15794860.001).
  • [ISSN] 1526-9655
  • [Journal-full-title] Clinical lymphoma
  • [ISO-abbreviation] Clin Lymphoma
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA 62242
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulin M
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82. Markotić A, Skerk V, Cvetko Krajinović L, Beus A: Is the clinical picture of Schnitzler syndrome always Schnitzler syndrome? Clin Exp Rheumatol; 2009 May-Jun;27(3):507-9
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  • Both patients had two major (monoclonal gammopathy and chronic urticaria) and almost all minor symptoms (e.g. arthralgia, bone pain, fever, etc.) of Schnitzler syndrome.
  • Our statement is that, besides clinical symptoms and signs, additional immune parameters should be tested before diagnosis of Schnitzler syndrome is established.
  • [MeSH-major] Schnitzler Syndrome / diagnosis. Schnitzler Syndrome / immunology
  • [MeSH-minor] CD4-Positive T-Lymphocytes / pathology. CD40 Ligand / blood. Diagnosis, Differential. Female. Granulocyte Colony-Stimulating Factor / blood. Humans. Interleukin-17 / blood. Interleukin-1beta / blood. Killer Cells, Natural / pathology. Male. Middle Aged. Urticaria / diagnosis. Urticaria / immunology. Urticaria / pathology

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  • (PMID = 19604447.001).
  • [ISSN] 0392-856X
  • [Journal-full-title] Clinical and experimental rheumatology
  • [ISO-abbreviation] Clin. Exp. Rheumatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / IL25 protein, human; 0 / Interleukin-17; 0 / Interleukin-1beta; 143011-72-7 / Granulocyte Colony-Stimulating Factor; 147205-72-9 / CD40 Ligand
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83. Hamidah NH, Azma RZ, Ezalia E, Das S, Umar NA, Swaminathan M, Mohamed Z, Abdul Wahid SF: Non-secretory multiple myeloma with diagnostic challenges. Clin Ter; 2010;161(5):445-8
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  • [Title] Non-secretory multiple myeloma with diagnostic challenges.
  • Non-secretory multiple myeloma (NSMM) is a rare variant of the classic form of multiple myeloma (MM).
  • In NSMM, no monoclonal gammopathy can be detected in serum or urine by conventional techniques, making the diagnosis more difficult.
  • Skeletal X-rays showed diffuse osteolytic lesions with multiple pathological fractures but there was no monoclonal gammopathy in the serum or urine.
  • The biopsy of the lytic lesion on the upper part of the femur showed infiltration by abnormal plasma cells.
  • A diagnosis of NSMM was made and he was treated with chemotherapy.
  • [MeSH-major] Multiple Myeloma / diagnosis

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  • (PMID = 20949241.001).
  • [ISSN] 1972-6007
  • [Journal-full-title] La Clinica terapeutica
  • [ISO-abbreviation] Clin Ter
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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84. Soubrier M: [POEMS syndrome]. Presse Med; 2007 Nov;36(11 Pt 2):1676-82
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  • The POEMS syndrome combines a constant polyneuropathy (P), organomegaly (O), endocrinopathy (E), monoclonal gammopathy (M) (or other plasma cell disorder) and skin changes (S).
  • Consistent plasma cell disorders include a monoclonal component, often in small quantities with a lambda light chain isotype, and plasmacytoma, often solitary lesions.
  • Treatment depends on specific characteristics of the disease and the patient (radiation therapy for plasmocytoma, autologous bone marrow transplantation in young subjects, corticosteroid therapy or chemotherapy in the elderly).

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  • (PMID = 17629447.001).
  • [ISSN] 0755-4982
  • [Journal-full-title] Presse medicale (Paris, France : 1983)
  • [ISO-abbreviation] Presse Med
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Number-of-references] 28
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85. Kimura S, Iwatsuka R, Aoki T, Odawara J, Asada N, Yamakura M, Takeuchi M, Matsue K: [Primary amyloidosis associated with IgD-lambda M-proteinemia]. Rinsho Ketsueki; 2007 Dec;48(12):1555-8
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  • We describe here a case of primary AL amyloidosis associated with IgD monoclonal gammopathy of undetermined significance.
  • Although serum electrophoresis revealed IgDlambda monoclonal protein, the bone marrow did not showed plasma cell proliferation.
  • A diagnosis of primary AL amyloidosis associated with IgD monoclonal gammopathy was made.
  • To our knowledge, this is the first report of primary AL amyloidosis associated with IgD monoclonal gammopathy with undetermined significance.

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  • (PMID = 18203516.001).
  • [ISSN] 0485-1439
  • [Journal-full-title] [Rinshō ketsueki] The Japanese journal of clinical hematology
  • [ISO-abbreviation] Rinsho Ketsueki
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Immunoglobulin D; 0 / Immunoglobulin lambda-Chains; 0 / Immunoglobulin mu-Chains
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86. Fend F: [Molecular pathology of plasma cell neoplasms]. Pathologe; 2010 Oct;31 Suppl 2:188-92
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  • [Title] [Molecular pathology of plasma cell neoplasms].
  • Plasma cell myeloma (PCM) and related immunosecretory disorders are a group of B-cell proliferations with a wide clinical and prognostic spectrum, characterized by the production of monoclonal immunoglobulin by immortalized plasma cells.
  • There is good evidence to support a multistep transformation process in plasma cell neoplasms, which corresponds to clinically discernible disease stages.
  • Monoclonal gammopathy of unknown significance is a common asymptomatic precursor lesion for PCM which carries an approximately 1% annual risk for progression.
  • Terminal disease stages are characterized by increasing genetic complexity and independence from bone marrow stromal cells and show a rapidly increasing tumour load with severe clinical symptoms.
  • Modern diagnostics of plasma cell neoplasms require inclusion of clinical, morphological, immunophenotypical and cytogenetic features to allow for individual risk assessment and therapy planning.
  • [MeSH-major] Neoplasms, Plasma Cell / genetics. Neoplasms, Plasma Cell / pathology
  • [MeSH-minor] Body Burden. Bone Marrow / pathology. Cell Transformation, Neoplastic / genetics. Cell Transformation, Neoplastic / pathology. DNA Mutational Analysis. Diagnosis, Differential. Disease Progression. Gene Expression Regulation, Neoplastic / genetics. Humans. In Situ Hybridization, Fluorescence. Neoplasm Staging. Paraproteinemias / genetics. Paraproteinemias / pathology. Plasmacytoma / genetics. Plasmacytoma / pathology. Risk Factors. Translocation, Genetic / genetics

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  • (PMID = 20852863.001).
  • [ISSN] 1432-1963
  • [Journal-full-title] Der Pathologe
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87. Golombick T, Diamond T: Prevalence of monoclonal gammopathy of undetermined significance/myeloma in patients with acute osteoporotic vertebral fractures. Acta Haematol; 2008;120(2):87-90
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  • [Title] Prevalence of monoclonal gammopathy of undetermined significance/myeloma in patients with acute osteoporotic vertebral fractures.


88. Eikenboom JC, Tjernberg P, Van Marion V, Heering KJ: Acquired von Willebrand syndrome: diagnostic problems and therapeutic options. Am J Hematol; 2007 Jan;82(1):55-8
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  • We present a case of acquired von Willebrand syndrome (AVWS) due to a monoclonal gammopathy of undetermined significance.
  • Initially this case was diagnosed as congenital von Willebrand disease (VWD); however, re-examination of the medical history rendered a congenital bleeding disorder unlikely.
  • A normal plasma von Willebrand factor (VWF) propeptide level and a very short half-life of VWF after a test infusion with factor VIII/VWF concentrate confirmed the diagnosis AVWS.
  • The differential diagnosis with congenital VWD and the diagnostic and therapeutic approaches of AVWS are discussed.
  • We conclude that the diagnosis of AVWS relies primarily on clinical suspicion and a careful bleeding history.
  • A correct diagnosis is essential for optimal perioperative management and treatment of bleeding episodes.
  • [MeSH-major] Hemorrhage / diagnosis. Hemorrhage / drug therapy. Immunoglobulins, Intravenous / administration & dosage. Immunologic Factors / administration & dosage. von Willebrand Diseases / diagnosis. von Willebrand Diseases / drug therapy
  • [MeSH-minor] Aged. Diagnosis, Differential. Humans. Male. Syndrome. von Willebrand Factor / analysis

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  • (PMID = 16986130.001).
  • [ISSN] 0361-8609
  • [Journal-full-title] American journal of hematology
  • [ISO-abbreviation] Am. J. Hematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulins, Intravenous; 0 / Immunologic Factors; 0 / von Willebrand Factor
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89. McMaster ML, Goldin LR, Bai Y, Ter-Minassian M, Boehringer S, Giambarresi TR, Vasquez LG, Tucker MA: Genomewide linkage screen for Waldenstrom macroglobulinemia susceptibility loci in high-risk families. Am J Hum Genet; 2006 Oct;79(4):695-701
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  • Waldenstrom macroglobulinemia (WM), a distinctive subtype of non-Hodgkin lymphoma that features overproduction of immunoglobulin M (IgM), clearly has a familial component; however, no susceptibility genes have yet been identified.
  • We performed a genomewide linkage analysis in 11 high-risk families with WM that were informative for linkage, for a total of 122 individuals with DNA samples, including 34 patients with WM and 10 patients with IgM monoclonal gammopathy of undetermined significance (IgM MGUS).
  • The strongest evidence of linkage was found on chromosomes 1q and 4q when patients with WM and with IgM MGUS were both considered affected; nonparametric linkage scores were 2.5 (P=.0089) and 3.1 (P=.004), respectively.

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  • (PMID = 16960805.001).
  • [ISSN] 0002-9297
  • [Journal-full-title] American journal of human genetics
  • [ISO-abbreviation] Am. J. Hum. Genet.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CP / N02-CP-01108; United States / Intramural NIH HHS / /
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, N.I.H., Intramural
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC1592553
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90. de Koning HD, Bodar EJ, van der Meer JW, Simon A, Schnitzler Syndrome Study Group: Schnitzler syndrome: beyond the case reports: review and follow-up of 94 patients with an emphasis on prognosis and treatment. Semin Arthritis Rheum; 2007 Dec;37(3):137-48
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  • OBJECTIVE: Schnitzler syndrome is a rare disorder characterized by a chronic urticarial rash and monoclonal gammopathy, accompanied by intermittent fever, arthralgia or arthritis, bone pain, and lymphadenopathy.
  • Our objectives are to systematically review disease characteristics of Schnitzler syndrome and collect follow-up information to gain insight into treatment efficacy and long-term prognosis.
  • METHODS: PubMed and MEDLINE databases (1966-2006) were searched, using the key words "Schnitzler syndrome," and the combination of "urticaria" with "monoclonal gammopathy," "immunoglobulin M (IgM)," or "paraproteinemia," as well as secondary references.
  • However, they had a 10-year risk of 15% of developing a lymphoproliferative disorder, most notably Waldenström's macroglobulinemia.
  • CONCLUSIONS: Schnitzler syndrome is a disabling disorder which affects multiple systems and which can be considered as an autoinflammatory syndrome.
  • There are new, effective treatment options, but close monitoring remains warranted because of the increased risk of lymphoproliferative disease.
  • [MeSH-minor] Diagnosis, Differential. Follow-Up Studies. Humans. Prognosis. Risk Factors

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  • [CommentIn] Semin Arthritis Rheum. 2008 Oct;38(2):163; author reply 164 [18304609.001]
  • (PMID = 17586002.001).
  • [ISSN] 0049-0172
  • [Journal-full-title] Seminars in arthritis and rheumatism
  • [ISO-abbreviation] Semin. Arthritis Rheum.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Number-of-references] 100
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91. Lottersberger F, Panza A, Lucchini G, Piatti S, Longhese MP: The Saccharomyces cerevisiae 14-3-3 proteins are required for the G1/S transition, actin cytoskeleton organization and cell wall integrity. Genetics; 2006 Jun;173(2):661-75
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  • [Title] The Saccharomyces cerevisiae 14-3-3 proteins are required for the G1/S transition, actin cytoskeleton organization and cell wall integrity.
  • To gain insights into the essential function(s) shared by these proteins, we searched for high-dosage suppressors of the growth defects of temperature-sensitive bmh mutants.
  • Both the protein kinase C1 (Pkc1) and its upstream regulators Wsc2 and Mid2 were found to act as high dosage suppressors of bmh mutants' temperature sensitivity, indicating a functional interaction between 14-3-3 and Pkc1.
  • Consistent with a role of 14-3-3 proteins in Pkc1-dependent cellular processes, shift to the restrictive temperature of bmh mutants severely impaired initiation of DNA replication, polarization of the actin cytoskeleton, and budding, as well as cell wall integrity.
  • Because Pkc1 acts in concert with the Swi4-Swi6 (SBF) transcriptional activator to control all these processes, the defective G(1)/S transition of bmh mutants might be linked to impaired SBF activity.
  • Indeed, the levels of the G(1) cyclin CLN2 transcripts, which are positively regulated by SBF, were dramatically reduced in bmh mutants.
  • Remarkably, budding and DNA replication defects of bmh mutants were suppressed by CLN2 expression from an SBF-independent promoter, suggesting that 14-3-3 proteins might contribute to regulating the late G(1) transcriptional program.
  • [MeSH-minor] Actins / metabolism. Base Sequence. Calcium-Binding Proteins / genetics. Calcium-Binding Proteins / metabolism. Cell Wall / metabolism. Cytoskeleton / metabolism. DNA, Fungal / genetics. G1 Phase. Gene Dosage. Genes, Fungal. Genes, Suppressor. Intracellular Signaling Peptides and Proteins. Membrane Glycoproteins. Membrane Proteins / genetics. Membrane Proteins / metabolism. Mutation. Osmosis. Protein Kinase C / genetics. Protein Kinase C / metabolism. S Phase. Temperature

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  • (PMID = 16648583.001).
  • [ISSN] 0016-6731
  • [Journal-full-title] Genetics
  • [ISO-abbreviation] Genetics
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / 14-3-3 Proteins; 0 / Actins; 0 / BMH1 protein, S cerevisiae; 0 / BMH2 protein, S cerevisiae; 0 / Calcium-Binding Proteins; 0 / DNA, Fungal; 0 / Intracellular Signaling Peptides and Proteins; 0 / MID2 protein, S cerevisiae; 0 / Membrane Glycoproteins; 0 / Membrane Proteins; 0 / Saccharomyces cerevisiae Proteins; 0 / WSC2 protein, S cerevisiae; EC 2.7.11.13 / PKC1 protein, S cerevisiae; EC 2.7.11.13 / Protein Kinase C
  • [Other-IDs] NLM/ PMC1526496
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92. Jagannath S: Value of serum free light chain testing for the diagnosis and monitoring of monoclonal gammopathies in hematology. Clin Lymphoma Myeloma; 2007 Sep;7(8):518-23
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  • [Title] Value of serum free light chain testing for the diagnosis and monitoring of monoclonal gammopathies in hematology.
  • The automated quantification of serum free kappa and lambda light chain concentrations provides a highly sensitive tool for the diagnosis and monitoring of monoclonal gammopathies.
  • An abnormal kappa:lambda ratio supports the presence of clonal plasma cell expansion and requires further investigation.
  • More than 94% of myeloma, light chain myeloma, and AL amyloidosis and, likewise, a majority of patients with light chain deposition disease are detectable with this technology.
  • Importantly, these assays identify M-proteins in most patients with oligosecretory disease and permit their recruitment into clinical trials from which they have been previously excluded.
  • Combining serum free light chain testing with traditional electrophoresis provides > 99% accuracy in the first-line diagnosis of monoclonal gammopathies and eliminates the need for urine testing in most instances.
  • One third of patients with monoclonal gammopathy of undetermined significance have an abnormal free light chain ratio, and these patients harbor a greater risk of progression to plasma cell dyscrasia.
  • Because the half-life of free light chains is < 6 hours, free light chain measurements at short sampling intervals allow real-time measurement of treatment-induced tumor kill, and provide prompt indications of chemosensitivity, dose adequacy, need for alternative approaches, and even prognosis, as demonstrated in AL amyloidosis if the involved free light chain concentration normalizes.
  • [MeSH-major] Immunoassay. Immunoglobulin kappa-Chains / blood. Immunoglobulin lambda-Chains / blood. Paraproteinemias / diagnosis
  • [MeSH-minor] Clinical Trials as Topic. Female. Hematology. Humans. Male. Plasmacytoma / diagnosis. Prognosis. Sensitivity and Specificity

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  • (PMID = 18021469.001).
  • [ISSN] 1557-9190
  • [Journal-full-title] Clinical lymphoma & myeloma
  • [ISO-abbreviation] Clin Lymphoma Myeloma
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulin kappa-Chains; 0 / Immunoglobulin lambda-Chains
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93. Cheung WI, Leung VK, Luk IS, Loke TK, Chan JC, Chau TN: Splenic tuberculosis associated with monoclonal gammopathy of undetermined significance and pericarditis. Hong Kong Med J; 2009 Aug;15(4):291-3
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  • [Title] Splenic tuberculosis associated with monoclonal gammopathy of undetermined significance and pericarditis.
  • Tuberculosis usually affects the respiratory system, but it may present atypically involving multiple systems, extrapulmonary systems, and manifest as a protein disorder.
  • Here we report a case of splenic tuberculosis associated with monoclonal gammopathy of undetermined significance, and pericarditis.
  • The diagnosis, confirmed by a plugged biopsy of the spleen, precluded the need for splenectomy in this patient and allowed prompt initiation of treatment, thereby avoiding the complications of tuberculous pericarditis and splenic infection.

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  • (PMID = 19652238.001).
  • [ISSN] 1024-2708
  • [Journal-full-title] Hong Kong medical journal = Xianggang yi xue za zhi
  • [ISO-abbreviation] Hong Kong Med J
  • [Language] ENG
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Antitubercular Agents; 0 / Biomarkers
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94. Fujimura T, Okuyama R, Ogawa E, Aiba S: Papuloerythroderma associated with monoclonal gammopathy of undetermined significance. J Dermatol; 2009 Apr;36(4):228-31
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  • [Title] Papuloerythroderma associated with monoclonal gammopathy of undetermined significance.
  • We describe a 73-year-old Japanese man with papuloerythroderma overlapped with monoclonal gammopathy of undetermined significance (MGUS).
  • A biochemical profile revealed the presence of immunoglobulin G kappa chain type monoclonal protein in the serum but the absence of hematological neoplasms.
  • We diagnosed the patient as papuloerythroderma with MGUS, and treated him with narrow-band ultraviolet B and topical steroid.
  • This case suggests an association between papuloerythroderma and MGUS.

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  • (PMID = 19348662.001).
  • [ISSN] 1346-8138
  • [Journal-full-title] The Journal of dermatology
  • [ISO-abbreviation] J. Dermatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Immunoglobulin G; 0 / Immunoglobulin kappa-Chains; 1A63Z067C8 / diflucortolone valerate; K253365DXI / Diflucortolone
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95. Martín-Aspas A, Ruiz-Blasco E, Atienza-Cuevas L, Durán-López MD: [Monoclonal gammopathy of undetermined significance and proteinaceous lymphadenopathy]. Med Clin (Barc); 2007 Jun 23;129(4):157-8
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  • [Title] [Monoclonal gammopathy of undetermined significance and proteinaceous lymphadenopathy].
  • [Transliterated title] Linfadenopatía proteínica asociada a gammapatía monoclonal de significado incierto.

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  • (PMID = 17663972.001).
  • [ISSN] 0025-7753
  • [Journal-full-title] Medicina clínica
  • [ISO-abbreviation] Med Clin (Barc)
  • [Language] spa
  • [Publication-type] Case Reports; Letter
  • [Publication-country] Spain
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96. Dear A, Brennan SO, Sheat MJ, Faed JM, George PM: Acquired dysfibrinogenemia caused by monoclonal production of immunoglobulin lambda light chain. Haematologica; 2007 Nov;92(11):e111-7
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  • [Title] Acquired dysfibrinogenemia caused by monoclonal production of immunoglobulin lambda light chain.
  • However, environmental and plasma factors can have an acquired effect on its expression or function.
  • Despite a low functional fibrinogen concentration and prolonged thrombin time, no inherited fibrinogen abnormality could be detected after extensive protein analysis and gene sequencing.
  • An immunoglobulin lambda light chain was found to bind fibrinogen as a monomer.
  • The 63-year-old patient acquired dysfibrinogenemia from a monoclonal production of lambda light chain that bound and inhibited the function of fibrinogen.
  • At age 64.5 he was diagnosed with monoclonal gammopathy of undetermined significance, explaining the abnormal immunoglobulin chain production.
  • [MeSH-minor] Fibrinogen / metabolism. Humans. Immunoglobulin lambda-Chains / metabolism. Male. Middle Aged. Monoclonal Gammopathy of Undetermined Significance / complications. Monoclonal Gammopathy of Undetermined Significance / diagnosis

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  • (PMID = 18024387.001).
  • [ISSN] 1592-8721
  • [Journal-full-title] Haematologica
  • [ISO-abbreviation] Haematologica
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Immunoglobulin lambda-Chains; 9001-32-5 / Fibrinogen
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97. Prignano F, Pescitelli L, Ricceri F, Ermini A, Lotti T: Development of monoclonal gammopathy in 12 patients receiving efalizumab treatment for chronic plaque psoriasis. J Am Acad Dermatol; 2010 Oct;63(4):e84-7
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  • [Title] Development of monoclonal gammopathy in 12 patients receiving efalizumab treatment for chronic plaque psoriasis.
  • [MeSH-major] Antibodies, Monoclonal / adverse effects. Paraproteinemias / chemically induced. Psoriasis / drug therapy

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  • [CommentIn] J Am Acad Dermatol. 2011 Jun;64(6):1184; author reply 1184-5 [21571182.001]
  • (PMID = 20846561.001).
  • [ISSN] 1097-6787
  • [Journal-full-title] Journal of the American Academy of Dermatology
  • [ISO-abbreviation] J. Am. Acad. Dermatol.
  • [Language] eng
  • [Publication-type] Letter
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / efalizumab
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98. Liu J, Hamrouni A, Wolowiec D, Coiteux V, Kuliczkowski K, Hetuin D, Saudemont A, Quesnel B: Plasma cells from multiple myeloma patients express B7-H1 (PD-L1) and increase expression after stimulation with IFN-{gamma} and TLR ligands via a MyD88-, TRAF6-, and MEK-dependent pathway. Blood; 2007 Jul 1;110(1):296-304
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  • [Title] Plasma cells from multiple myeloma patients express B7-H1 (PD-L1) and increase expression after stimulation with IFN-{gamma} and TLR ligands via a MyD88-, TRAF6-, and MEK-dependent pathway.
  • Multiple myeloma (MM) cells inhibit certain T-cell functions.
  • We examined the expression of B7-H1 (PD-L1), a B7-related protein that inhibits T-cell responses, in CD138-purified plasma cells isolated from MM patients, monoclonal gammopathy of undetermined significance patients, and healthy donors.
  • We observed that B7-H1 was expressed in most MM plasma cells, but not cells isolated from monoclonal gammopathy of undetermined significance or healthy donors.
  • This expression was increased or induced by IFN-gamma and Toll-like receptor (TLR) ligands in isolated MM plasma cells.
  • MM plasma cells stimulated with IFN-gamma or TLR ligands inhibited cytotoxic T lymphocytes (CTLs) generation and this immunosuppressive effect was inhibited by preincubation with an anti-B7-H1 antibody, the UO126 MEK inhibitor, or by transfection of a dominant-negative mutant of MyD88.
  • [MeSH-minor] Antigens, CD274. Case-Control Studies. Humans. Ligands. Mitogen-Activated Protein Kinase Kinases / metabolism. Myeloid Differentiation Factor 88 / metabolism. Neoplasm Proteins / analysis. Neoplasm Proteins / genetics. Plasma Cells / chemistry. Plasma Cells / pathology. T-Lymphocytes, Cytotoxic / drug effects. TNF Receptor-Associated Factor 6 / metabolism. Tumor Cells, Cultured. Up-Regulation / drug effects. Up-Regulation / genetics

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  • (PMID = 17363736.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Antigens, CD274; 0 / CD274 protein, human; 0 / Ligands; 0 / MYD88 protein, human; 0 / Myeloid Differentiation Factor 88; 0 / Neoplasm Proteins; 0 / TNF Receptor-Associated Factor 6; 0 / Toll-Like Receptors; 82115-62-6 / Interferon-gamma; EC 2.7.12.2 / Mitogen-Activated Protein Kinase Kinases
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99. Sakurai-Chin C, Ubara Y, Suwabe T, Hoshino J, Yonaha T, Hasegawa E, Sumida K, Hiramatsu R, Yamanouchi M, Hayami N, Yamauchi J, Tominaga N, Sawa N, Takemoto F, Masuoka K, Takaichi K, Oohashi K: AL amyloidosis with IgD-lambda monoclonal gammopathy and lambda-type Bence-Jones protein: successful treatment by autologous stem cell transplantation. Clin Exp Nephrol; 2010 Oct;14(5):506-10
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  • [Title] AL amyloidosis with IgD-lambda monoclonal gammopathy and lambda-type Bence-Jones protein: successful treatment by autologous stem cell transplantation.
  • A 45-year-old Japanese woman had been diagnosed with monoclonal gammopathy of undetermined significance (MGUS) featuring urinary Bence-Jones protein of the lambda type (BJP-lambda) for 11 years.
  • She was diagnosed as having AL amyloidosis with IgD-lambda monoclonal gammopathy and BJP-lambda.
  • Autologous stem cell transplantation (SCT) was done after chemotherapy with vincristine, daunorubicin, dexamethasone (VAD), and high-dose melphalan (HDM).
  • [MeSH-major] Amyloidosis / surgery. Bence Jones Protein / urine. Hematopoietic Stem Cell Transplantation. Immunoglobulin D / immunology. Paraproteinemias / surgery. Transplantation, Autologous

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  • (PMID = 20632063.001).
  • [ISSN] 1437-7799
  • [Journal-full-title] Clinical and experimental nephrology
  • [ISO-abbreviation] Clin. Exp. Nephrol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Immunoglobulin D; 9006-99-9 / Bence Jones Protein
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100. Faiman B, Licata AA: New tools for detecting occult monoclonal gammopathy, a cause of secondary osteoporosis. Cleve Clin J Med; 2010 Apr;77(4):273-8
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  • [Title] New tools for detecting occult monoclonal gammopathy, a cause of secondary osteoporosis.
  • Most patients with multiple myeloma or other monoclonal gammopathies present with anemia, hypercalcemia, or renal insufficiency.
  • Measuring the concentration and ratio of free light chains in the serum can help detect monoclonal gammopathy and help to differentiate myeloma-related bone loss from other secondary forms of osteoporosis.
  • [MeSH-major] Back Pain / diagnosis. Back Pain / etiology. Monoclonal Gammopathy of Undetermined Significance / complications. Monoclonal Gammopathy of Undetermined Significance / diagnosis. Multiple Myeloma / diagnosis. Osteoporosis / diagnosis. Osteoporosis / etiology. Spinal Fractures / diagnosis. Spinal Fractures / etiology
  • [MeSH-minor] Absorptiometry, Photon. Aged, 80 and over. Bone Density. Bone Resorption. Diagnosis, Differential. Female. Humans. Male. Middle Aged






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