[X] Close
You are about to erase all the values you have customized, search history, page format, etc.
Click here to RESET all values       Click here to GO BACK without resetting any value
Items 1 to 21 of about 21
1. Dowdell KC, Pesnicak L, Hoffmann V, Steadman K, Remaley AT, Cohen JI, Straus SE, Rao VK: Valproic acid (VPA), a histone deacetylase (HDAC) inhibitor, diminishes lymphoproliferation in the Fas -deficient MRL/lpr(-/-) murine model of autoimmune lymphoproliferative syndrome (ALPS). Exp Hematol; 2009 Apr;37(4):487-94
PDF icon [Fulltext service] Download fulltext PDF of this article and others, as many as you want.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Valproic acid (VPA), a histone deacetylase (HDAC) inhibitor, diminishes lymphoproliferation in the Fas -deficient MRL/lpr(-/-) murine model of autoimmune lymphoproliferative syndrome (ALPS).
  • OBJECTIVE: Autoimmune lymphoproliferative syndrome (ALPS) is a disorder of apoptosis, often presenting in childhood.
  • Similarly, MRL/lpr(-/-) mice homozygous for Fas mutations develop an ALPS-like disease with autoimmunity, lymphadenopathy, splenomegaly, and expansion of double-negative T cells.
  • Currently, there are no proven therapies with adequate safety margins for sustained abolition of the lymphoproliferation associated with ALPS.
  • MATERIALS AND METHODS: Human peripheral blood mononuclear cells from patients with ALPS and normal controls were tested in vitro to determine the efficacy of VPA at inducing cell death.
  • VPA was used in vivo to control lymphoproliferation in MRL/lpr(-/-) mice, a model for ALPS.
  • CONCLUSION: Based on our data, VPA is effective at reducing lymphoproliferation in mice, and is currently being studied in a clinical trial as a lympholytic agent in patients with ALPS.
  • [MeSH-major] Apoptosis / drug effects. Autoimmune Diseases / drug therapy. Enzyme Inhibitors / pharmacology. Histone Deacetylase Inhibitors. Lymphoproliferative Disorders / drug therapy. T-Lymphocytes / drug effects. Valproic Acid / pharmacology
  • [MeSH-minor] Animals. Antigens, CD95 / genetics. Cell Proliferation / drug effects. Cells, Cultured. Disease Models, Animal. Female. Humans. Leukocytes, Mononuclear / cytology. Leukocytes, Mononuclear / drug effects. Leukocytes, Mononuclear / immunology. Mice. Mice, Knockout. Reference Standards. Syndrome


2. Michiels JJ, Budde U, van der Planken M, van Vliet HH, Schroyens W, Berneman Z: Acquired von Willebrand syndromes: clinical features, aetiology, pathophysiology, classification and management. Best Pract Res Clin Haematol; 2001 Jun;14(2):401-36
PDF icon [Fulltext service] Get downloadable fulltext PDFs of articles closely matching to this article, as many as you want.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Acquired von Willebrand syndrome (AVWS) associated with hypothyroidism is of type I, results from a decreased synthesis of factor VIII and von Willebrand factor (VWF), responds to desmopressin with normal half-life times for factor VIII and VWF parameters, and disappears after treatment with I-thyroxine.
  • AVWS type I or III, which occurs in a minority of patients with Wilms' tumour in the complete absence of an inhibitor against VWF and no absorption of factor VIII or VWF onto nephroblastoma cells, responds to chemotherapy and/or tumour resection.
  • AVWS associated with thrombocythaemia of various myeloproliferative disorders is characterized by normal factor VIII and von Willebrand factor antigen (VWF: Ag) levels and a selective deficiency of functional ristocetin co-factor activity (VWF: RCo) and collagen-binding activity (VWF: CBA).
  • AVWS type II in thrombocythaemia is caused by a platelet-dependent proteolysis of large VWF multimers, given the inverse relationship between platelet count and large VWF multimers in plasma and specific increases in the number of proteolytic VWF fragments in plasma.
  • The laboratory findings of AVWS associated with systemic lupus erythematosus or IgG benign monoclonal gammopathy are characterized by a prolonged bleeding time and activated partial thromboplastin time, decreased or absent ristocetin-induced platelet activity, low to very low levels of factor VIII coagulant activity (mean 15%), VWF: Ag (mean 10.7%) and VWF: RCo (mean 6.2%), and a type II multimeric pattern of VWF.
  • High-dose intravenous immunoglobulin transiently corrects the factor VIII coagulant and VWF levels, lasting for a few weeks in AVWS type II associated with systemic lupus erythematosus or IgG benign monoclonal gammopathy.
  • Prednisolone is effective in AVWS associated with autoimmune disorder.
  • Prednisolone and chemotherapy will not affect AVWS associated with IgG benign monoclonal gammopathy because the monoclonal IgG protein remains to act as an anti-VWF autoantibody.
  • An absorption of VWF to malignant cells has been documented in a few patients with various lymphoproliferative disorders or adrenal carcinoma and suggested to result in a depletion of VWF.
  • The clinical picture of AVWS associated with early-stage IgG multiple myeloma, chronic lymphocytic leukaemia or non-Hodgkin's lymphoma without a paraprotein or no detectable underlying disorder is similar to that of AVWS type II in IgG benign monoclonal gammopathy but poorly documented with regard to the underlying immune mechanism of AVWS.
  • Drug-induced AVWS has been described in association with the use of pesticides valproic acid, ciprofloxacin, griseofulvin, tetracycline, thrombolytic agents and hydroxyethyl starch.
  • [MeSH-minor] Hematologic Tests. Humans. Kidney Neoplasms / complications. Lupus Erythematosus, Systemic / complications. Lymphoproliferative Disorders / complications. Paraproteinemias / complications. Syndrome. Wilms Tumor / complications

  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 11686107.001).
  • [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] 148
  •  go-up   go-down


3. Ramos-Casals M, De Vita S, Tzioufas AG: Hepatitis C virus, Sjögren's syndrome and B-cell lymphoma: linking infection, autoimmunity and cancer. Autoimmun Rev; 2005 Jan;4(1):8-15
PDF icon [Fulltext service] Get downloadable fulltext PDFs of articles closely matching to this article, as many as you want.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Hepatitis C virus, Sjögren's syndrome and B-cell lymphoma: linking infection, autoimmunity and cancer.
  • An increased prevalence of hematologic malignancies is often described in patients with Sjögren's syndrome (SS).
  • Viruses have been proposed as possible etiologic or triggering agents of systemic autoimmune diseases (SADs), with hepatitis C virus (HCV) being one of the viruses most frequently associated with autoimmune features and with systemic autoimmune diseases such as mixed cryoglobulinemia or SS.
  • Moreover, the association between HCV infection and hematologic malignancies, mainly non-Hodgkin's lymphoma (NHL), is supported by several studies.
  • For these reasons, the recognized association of specific systemic autoimmune diseases (mainly SS and mixed cryoglobulinemia) with HCV infection, added to the possible evolution of any one of these entities into a B-cell NHL, suggests the possibility of a close relationship among SS, HCV and B-cell lymphoproliferative disorders, especially in patients with type II mixed cryoglobulinemia.
  • [MeSH-major] Autoimmunity / immunology. Hepacivirus / immunology. Hepatitis C / immunology. Lymphoma, B-Cell / immunology. Sjogren's Syndrome / immunology

  • Genetic Alliance. consumer health - C Syndrome.
  • Genetic Alliance. consumer health - Hepatitis.
  • MedlinePlus Health Information. consumer health - Hepatitis C.
  • MedlinePlus Health Information. consumer health - Sjogren's Syndrome.
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 15652773.001).
  • [ISSN] 1568-9972
  • [Journal-full-title] Autoimmunity reviews
  • [ISO-abbreviation] Autoimmun Rev
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Autoantigens; 0 / Ribonucleoproteins; 0 / SS-A antigen; 0 / SS-B antigen
  • [Number-of-references] 32
  •  go-up   go-down


Advertisement
4. Hong YH, Lee CK: Autoimmune lymphoproliferative syndrome-like syndrome presented as lupus-like syndrome with mycobacterial joint infection evolved into the lymphoma. Rheumatol Int; 2009 Mar;29(5):569-73
PDF icon [Fulltext service] Get downloadable fulltext PDFs of articles closely matching to this article, as many as you want.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Autoimmune lymphoproliferative syndrome-like syndrome presented as lupus-like syndrome with mycobacterial joint infection evolved into the lymphoma.
  • The autoimmune lymphoproliferative syndrome (ALPS) and ALPS-like syndrome are variable clinical conditions characterized by lymphoproliferative disease, autoimmune cytopenias and susceptibility to malignancy.
  • A low-grade fever, intermittent hypotension and confusion were associated with the pain.
  • The patient was treated with anti-mycobacterial drugs, NSAIDs and glucocorticoids for 10 months.
  • But with the symptoms worsening, the patient developed cervical lymph node enlargements and was diagnosed as a diffuse large B cell lymphoma with hemophagocytosis on biopsy.
  • [MeSH-major] Autoimmune Diseases / pathology. Joints / pathology. Lymphoma, Large B-Cell, Diffuse / pathology. Lymphoproliferative Disorders / pathology. Mycobacterium Infections / pathology
  • [MeSH-minor] Anti-Bacterial Agents / therapeutic use. Anti-Inflammatory Agents, Non-Steroidal / therapeutic use. Female. Glucocorticoids / therapeutic use. Humans. Lupus Erythematosus, Systemic / pathology. Middle Aged. Syndrome


5. Mascia MT, Ferrari D, Campioli D, Sandri G, Mussini C, Ferri C: Non HCV-related mixed cryoglobulinemia. Dig Liver Dis; 2007 Sep;39 Suppl 1:S61-4
PDF icon [Fulltext service] Get downloadable fulltext PDFs of articles closely matching to this article, as many as you want.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Interestingly, patients with Sjögren's syndrome or lymphoma had higher levels of cryocrit with cryoglobulinemic syndrome comparable to that found in HCV-positive MC patients.
  • MC is a multifactorial disorder; considering possible etiological factors and clinical associations the disease may present different subsets: the prevalent group of HCV-positive MC; HCV-positive MC associated with different autoimmune lymphoproliferative disorders; two MC subsets without any apparent causative agent: those with well-known autoimmune lymphoproliferative disorders and the rare cases of "essential" MC; and finally MC associated with other infectious agents.

  • Genetic Alliance. consumer health - Cryoglobulinemia.
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 17936226.001).
  • [ISSN] 1878-3562
  • [Journal-full-title] Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver
  • [ISO-abbreviation] Dig Liver Dis
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Netherlands
  •  go-up   go-down


6. Manoussakis MN, Moutsopoulos HM: Sjögren's syndrome: autoimmune epithelitis. Baillieres Best Pract Res Clin Rheumatol; 2000 Mar;14(1):73-95
PDF icon [Fulltext service] Get downloadable fulltext PDFs of articles closely matching to this article, as many as you want.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Sjögren's syndrome: autoimmune epithelitis.
  • Sjögren's syndrome (SS) is a chronic autoimmune disorder of the exocrine glands of unknown aetiology, which is typically associated with focal lymphocytic infiltrates of glandular tissues and autoantibody responses against the Ro(SSA) and La(SSB) ribonucleoproteins.
  • In almost one-third of patients disease involves various extraglandular sites, whereas approximately 5% of patients may also develop malignant B-cell lymphoma.
  • In addition, features of SS are frequently encountered (5-20%) in patients with several other autoimmune rheumatic diseases, and in several respects these 'secondary' forms may be distinct from SS found alone (primary-SS), as well as from each other.
  • Differential diagnosis includes adverse effects of drugs, sarcoidosis, lipoproteinaemias, age-related atrophy, chronic graft-versus-host disease, lymphomas, amyloidosis and infection by human immunodeficiency virus or hepatitis C virus.
  • [MeSH-major] Sjogren's Syndrome / diagnosis. Sjogren's Syndrome / therapy
  • [MeSH-minor] Diagnosis, Differential. Humans. Immune System / physiopathology. Lymphoproliferative Disorders / etiology

  • MedlinePlus Health Information. consumer health - Sjogren's Syndrome.
  • NCI CPTC Antibody Characterization Program. NCI CPTC Antibody Characterization Program .
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 10882215.001).
  • [Journal-full-title] Baillière's best practice & research. Clinical rheumatology
  • [ISO-abbreviation] Baillieres Best Pract Res Clin Rheumatol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] ENGLAND
  •  go-up   go-down


7. Yeh S, Li Z, Sen HN, Lim WK, Gill F, Perkins K, Rao VK, Nussenblatt RB: Scleritis and multiple systemic autoimmune manifestations in chronic natural killer cell lymphocytosis associated with elevated TCRalpha/beta+CD3+CD4-CD8- double-negative T cells. Br J Ophthalmol; 2010 Jun;94(6):748-52
PDF icon [Fulltext service] Download fulltext PDF of this article and others, as many as you want.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Scleritis and multiple systemic autoimmune manifestations in chronic natural killer cell lymphocytosis associated with elevated TCRalpha/beta+CD3+CD4-CD8- double-negative T cells.
  • BACKGROUND/AIMS: Chronic natural killer lymphocytosis (CNKL) has been associated with systemic autoimmunity; however, its association with scleritis or ocular autoimmunity has not been characterised.
  • The natural killer (NK) cell function and immunophenotype of a patient with CNKL who developed bilateral scleritis and multiple systemic autoimmune findings were evaluated.
  • RESULTS: A 56-year-old woman with vitiligo, psoriatic arthritis, thyroiditis, erythema nodosum, bilateral anterior scleritis and Sjogren syndrome was managed with multiple immunosuppressive medications, including prednisone, mycophenolate mofetil and methotrexate.
  • An abnormal elevation of TCRalpha/beta(+) CD3(+)CD4(-)CD8(-) T cells suggestive of autoimmune lymphoproliferative syndrome was observed; however, apoptosis dysfunction was not found.
  • [MeSH-major] Autoimmune Diseases / immunology. Killer Cells, Natural / immunology. Lymphocytosis / immunology. Scleritis / immunology. T-Lymphocyte Subsets / immunology

  • MedlinePlus Health Information. consumer health - Autoimmune Diseases.
  • NCI CPTC Antibody Characterization Program. NCI CPTC Antibody Characterization Program .
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • [Cites] Clin Immunol. 2002 Jul;104(1):21-30 [12139944.001]
  • [Cites] Autoimmun Rev. 2008 May;7(5):384-90 [18486926.001]
  • [Cites] Br J Dermatol. 2003 Jul;149(1):160-4 [12890211.001]
  • [Cites] J Autoimmun. 2003 Aug;21(1):83-92 [12892739.001]
  • [Cites] Ophthalmology. 2004 Mar;111(3):501-6 [15019326.001]
  • [Cites] Brain. 2004 Sep;127(Pt 9):1917-27 [15229129.001]
  • [Cites] Arthritis Rheum. 1983 Aug;26(8):954-60 [6882489.001]
  • [Cites] Ophthalmology. 1985 Nov;92(11):1542-9 [3878485.001]
  • [Cites] Arch Dermatol Res. 1986;278(4):264-9 [3740937.001]
  • [Cites] Clin Exp Immunol. 1988 May;72(2):299-302 [3409548.001]
  • [Cites] Cancer. 1989 Jan 1;63(1):90-5 [2783378.001]
  • [Cites] J Neurol Sci. 1989 Dec;94(1-3):115-23 [2614463.001]
  • [Cites] Ophthalmology. 1991 Apr;98(4):472-9 [1828871.001]
  • [Cites] Blood. 1994 Oct 15;84(8):2721-5 [7919384.001]
  • [Cites] Leuk Lymphoma. 1996 Jan;20(3-4):245-8 [8624463.001]
  • [Cites] Blood. 1997 Feb 15;89(4):1341-8 [9028957.001]
  • [Cites] Am J Pathol. 1997 Feb;150(2):653-66 [9033278.001]
  • [Cites] J Neuroimmunol. 1998 Jun 15;86(2):123-33 [9663557.001]
  • [Cites] J Biol Chem. 2005 Feb 11;280(6):4772-8 [15563472.001]
  • [Cites] J Immunol. 2005 May 1;174(9):5187-91 [15843513.001]
  • [Cites] Intern Med. 2007;46(5):251-4 [17329922.001]
  • [Cites] Immunol Res. 2008;40(1):87-92 [18193364.001]
  • [Cites] Br J Ophthalmol. 2008 Mar;92(3):417-9 [18303163.001]
  • [Cites] Nat Immunol. 2002 Sep;3(9):807-13 [12205470.001]
  • (PMID = 20508050.001).
  • [ISSN] 1468-2079
  • [Journal-full-title] The British journal of ophthalmology
  • [ISO-abbreviation] Br J Ophthalmol
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / / Z01 EY000393-06; United States / Intramural NIH HHS / / ZIA EY000376-09
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Cytokines; 0 / Interleukin-2; 0 / Receptors, Antigen, T-Cell, alpha-beta
  • [Other-IDs] NLM/ NIHMS320628; NLM/ PMC3172679
  •  go-up   go-down


8. De Re V, De Vita S, Gasparotto D, Marzotto A, Carbone A, Ferraccioli G, Boiocchi M: Salivary gland B cell lymphoproliferative disorders in Sjögren's syndrome present a restricted use of antigen receptor gene segments similar to those used by hepatitis C virus-associated non-Hodgkins's lymphomas. Eur J Immunol; 2002 Mar;32(3):903-10
PDF icon [Fulltext service] Download fulltext PDF of this article and others, as many as you want.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Salivary gland B cell lymphoproliferative disorders in Sjögren's syndrome present a restricted use of antigen receptor gene segments similar to those used by hepatitis C virus-associated non-Hodgkins's lymphomas.
  • Sjögren's syndrome (SS) represents a pathological model of the evolution from polyclonal B lymphocyte activation to oligoclonal/monoclonal B cell expansion, which may culminate in the development of a malignant lymphoproliferative disease.
  • However, the agent(s) able to trigger B cell proliferation is still unknown.
  • A similar pathogenetic mechanism exist in mixed cryoglobulinemia, another autoimmune disease that often evolves to non-Hodgkin's lymphoma (NHL) and in which hepatitis C virus (HCV) infection has been demonstrated to play an etiopathogenetic role.
  • In the present study, we cloned and sequenced the antigen receptor (IgR) variable region genes of SS-associated monoclonal non-neoplastic lymphoproliferations and compared them with those of our previous reported HCV-associated NHL, to derive clues on the antigen(s) that sustains SS.
  • In conclusion, although there are significant differences between SS and HCV-associated lymphoproliferative diseases, they share many molecular characteristics, which suggest an immunological cross-reactivity or molecular mimicry among the agents that underlie these disorders.
  • [MeSH-major] Autoimmune Diseases / complications. Gene Rearrangement, B-Lymphocyte. Lymphoma, B-Cell / etiology. Lymphoproliferative Disorders / etiology. Parotid Neoplasms / etiology. Receptors, Antigen, B-Cell / genetics. Sjogren's Syndrome / complications
  • [MeSH-minor] Adult. Aged. Amino Acid Sequence. Autoantibodies / immunology. Cell Transformation, Neoplastic. Clone Cells / immunology. Clone Cells / pathology. Complementarity Determining Regions / genetics. Cross Reactions. Disease Progression. Female. Genes, Immunoglobulin. Hepacivirus / isolation & purification. Humans. Immunoglobulin Heavy Chains / genetics. Immunoglobulin Variable Region / genetics. Immunoglobulin kappa-Chains / genetics. Lymphocyte Activation. Lymphoma, Non-Hodgkin / genetics. Lymphoma, Non-Hodgkin / immunology. Lymphoma, Non-Hodgkin / virology. Male. Middle Aged. Molecular Mimicry. Molecular Sequence Data. Polymerase Chain Reaction. Rheumatoid Factor / immunology. Sequence Alignment. Sequence Homology, Amino Acid. Sialadenitis / immunology. Sialadenitis / pathology

  • Genetic Alliance. consumer health - C Syndrome.
  • Genetic Alliance. consumer health - Hepatitis.
  • Genetic Alliance. consumer health - B-Cell Lymphomas.
  • MedlinePlus Health Information. consumer health - Autoimmune Diseases.
  • MedlinePlus Health Information. consumer health - Sjogren's Syndrome.
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 11870635.001).
  • [ISSN] 0014-2980
  • [Journal-full-title] European journal of immunology
  • [ISO-abbreviation] Eur. J. Immunol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Autoantibodies; 0 / Complementarity Determining Regions; 0 / Immunoglobulin Heavy Chains; 0 / Immunoglobulin Variable Region; 0 / Immunoglobulin kappa-Chains; 0 / Receptors, Antigen, B-Cell; 9009-79-4 / Rheumatoid Factor
  •  go-up   go-down


9. Rao VK, Price S, Perkins K, Aldridge P, Tretler J, Davis J, Dale JK, Gill F, Hartman KR, Stork LC, Gnarra DJ, Krishnamurti L, Newburger PE, Puck J, Fleisher T: Use of rituximab for refractory cytopenias associated with autoimmune lymphoproliferative syndrome (ALPS). Pediatr Blood Cancer; 2009 Jul;52(7):847-52
PDF icon [Fulltext service] Download fulltext PDF of this article and others, as many as you want.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Use of rituximab for refractory cytopenias associated with autoimmune lymphoproliferative syndrome (ALPS).
  • BACKGROUND: ALPS is a disorder of apoptosis resulting in accumulation of autoreactive lymphocytes, leading to marked lymphadenopathy, hepatosplenomegaly, and multilineage cytopenias due to splenic sequestration and/or autoimmune destruction often presenting in childhood.
  • We summarize our experience of rituximab use during the last 8 years in 12 patients, 9 children, and 3 adults, out of 259 individuals with ALPS, belonging to 166 families currently enrolled in studies at the National Institutes of Health.
  • METHODS: Refractory immune thrombocytopenia (platelet count <20,000) in nine patients and autoimmune hemolytic anemia (AIHA) in three patients led to treatment with rituximab.
  • RESULTS: In seven out of nine patients with ALPS and thrombocytopenia, rituximab therapy led to median response duration of 21 months (range 14-36 months).
  • Noted toxicities included profound and prolonged hypogammaglobulinemia in three patients requiring replacement IVIG, total absence of antibody response to polysaccharide vaccines lasting up to 4 years after rituximab infusions in one patient and prolonged neutropenia in one patient.
  • CONCLUSION: Toxicities including hypogammaglobulinemia and neutropenia constitute an additional infection risk burden, especially in asplenic individuals, and may warrant avoidance of rituximab until other immunosuppressive medication options are exhausted.
  • Long-term follow-up of ALPS patients with cytopenias after any treatment is necessary to determine relative risks and benefits.

  • Genetic Alliance. consumer health - Autoimmune Lymphoproliferative Syndrome.
  • MedlinePlus Health Information. consumer health - Autoimmune Diseases.
  • COS Scholar Universe. author profiles.
  • ClinicalTrials.gov. clinical trials - ClinicalTrials.gov .
  • Hazardous Substances Data Bank. RITUXIMAB .
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • [Copyright] (c) 2009 Wiley-Liss, Inc.
  • [Cites] Leuk Lymphoma. 2001 May;41(5-6):501-11 [11378568.001]
  • [Cites] Blood. 2001 Jul 1;98(1):194-200 [11418480.001]
  • [Cites] Blood. 2001 Aug 15;98(4):952-7 [11493438.001]
  • [Cites] Blood. 2001 Oct 15;98(8):2466-73 [11588044.001]
  • [Cites] Br J Haematol. 2002 Apr;117(1):176-88 [11918552.001]
  • [Cites] Br J Haematol. 2002 Sep;118(4):1078-81 [12199788.001]
  • [Cites] Nature. 2002 Sep 26;419(6905):395-9 [12353035.001]
  • [Cites] Blood. 2003 May 15;101(10):3857-61 [12531800.001]
  • [Cites] Curr Opin Rheumatol. 2003 Jul;15(4):417-21 [12819469.001]
  • [Cites] N Engl J Med. 2004 Sep 30;351(14):1409-18 [15459302.001]
  • [Cites] J Pediatr. 1967 Jun;70(6):891-9 [4165068.001]
  • [Cites] J Clin Invest. 1992 Aug;90(2):334-41 [1386609.001]
  • [Cites] Science. 1995 Jun 2;268(5215):1347-9 [7539157.001]
  • [Cites] Cell. 1995 Jun 16;81(6):935-46 [7540117.001]
  • [Cites] Blood. 1997 Feb 15;89(4):1341-8 [9028957.001]
  • [Cites] Am J Pathol. 1998 Nov;153(5):1541-50 [9811346.001]
  • [Cites] Ann Intern Med. 1999 Apr 6;130(7):591-601 [10189330.001]
  • [Cites] Am J Hum Genet. 1999 Apr;64(4):1002-14 [10090885.001]
  • [Cites] Br J Haematol. 2005 May;129(4):534-8 [15877736.001]
  • [Cites] Hum Genet. 2006 Apr;119(3):284-94 [16446975.001]
  • [Cites] Blood. 2006 Apr 1;107(7):2639-42 [16352811.001]
  • [Cites] Blood. 2006 Sep 15;108(6):1965-71 [16757690.001]
  • [Cites] J Pediatr Hematol Oncol. 2006 Dec;28(12):824-6 [17164652.001]
  • [Cites] J Pediatr. 2007 Apr;150(4):338-44, 344.e1 [17382107.001]
  • [Cites] Br J Haematol. 2007 May;137(4):349-54 [17456057.001]
  • [Cites] Proc Natl Acad Sci U S A. 2007 May 22;104(21):8953-8 [17517660.001]
  • [Cites] BMC Med Genet. 2007;8:41 [17605793.001]
  • [Cites] Eur J Haematol. 2007 Oct;79(4):363-6 [17725802.001]
  • [Cites] Arthritis Rheum. 2007 Sep;56(9):3044-56 [17763423.001]
  • [Cites] Am J Hematol. 2007 Dec;82(12):1049-55 [17674358.001]
  • [Cites] Blood. 2008 Jan 15;111(2):705-14 [17925488.001]
  • [Cites] Br J Haematol. 2008 Apr;141(2):149-69 [18318765.001]
  • [Cites] Pathology. 2008 Apr;40(3):288-94 [18428049.001]
  • [Cites] Blood. 2008 Jul 15;112(2):277-86 [18319398.001]
  • [Cites] Pediatr Blood Cancer. 2009 Feb;52(2):259-62 [18937333.001]
  • [Cites] Exp Hematol. 2009 Apr;37(4):487-94 [19217201.001]
  • (PMID = 19214977.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] ENG
  • [Grant] United States / CCR NIH HHS / RC / HHSN261200800001C; United States / NCI NIH HHS / CA / HHSN261200800001E; United States / Intramural NIH HHS / / Z99 AI999999; United States / PHS HHS / / HHSN261200800001E
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, N.I.H., Intramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Murine-Derived; 0 / Immunosuppressive Agents; 4F4X42SYQ6 / Rituximab
  • [Other-IDs] NLM/ NIHMS123735; NLM/ PMC2774763
  •  go-up   go-down


10. Müllauer L, Gruber P, Sebinger D, Buch J, Wohlfart S, Chott A: Mutations in apoptosis genes: a pathogenetic factor for human disease. Mutat Res; 2001 Jul;488(3):211-31
PDF icon [Fulltext service] Get downloadable fulltext PDFs of articles closely matching to this article, as many as you want.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Cell death by apoptosis is exerted by the coordinated action of many different gene products.
  • Mutations in the homologous receptor Fas (also named CD95; Apo-1) are observed in malignant lymphomas, solid tumors and the autoimmune lymphoproliferative syndrome type I (ALPS I).
  • Caspase 10, a representative of the caspase family of proteases, which plays a central role in the execution of apoptosis, is defect in autoimmune lymphoproliferative syndrome type II (ALPS II).
  • The intracellular pro-apoptotic molecule bcl-10 is frequently mutated in mucosa-associated lymphoid tissue (MALT) lymphomas and various non-hematologic malignancies.
  • Anti-apoptotic proteins like bcl-2, cellular-inhibitor of apoptosis protein 2 (c-IAP2) and neuronal apoptosis inhibitory protein 1 (NAIP1) are often altered in follicular lymphomas, MALT lymphomas and spinal muscular atrophy (SMA), respectively.
  • [MeSH-major] Adaptor Proteins, Signal Transducing. Apoptosis / genetics. Autoimmune Diseases / genetics. Neoplasms / genetics
  • [MeSH-minor] Antigens, CD95 / genetics. Antigens, CD95 / physiology. Caspases / genetics. Caspases / physiology. Cysteine Proteinase Inhibitors / genetics. Cysteine Proteinase Inhibitors / physiology. Drug Design. Fas Ligand Protein. Forecasting. Genes, bcl-2. Genes, p53. Humans. Membrane Glycoproteins / genetics. Membrane Glycoproteins / physiology. Models, Biological. Mutation. Neoplasm Proteins / genetics. Neoplasm Proteins / physiology. Nerve Tissue Proteins / genetics. Nerve Tissue Proteins / physiology. Neuronal Apoptosis-Inhibitory Protein. Perforin. Pore Forming Cytotoxic Proteins. Prognosis. Protein Structure, Tertiary. Proto-Oncogene Proteins / genetics. Proto-Oncogene Proteins / physiology. Proto-Oncogene Proteins c-bcl-2 / physiology. Receptors, Tumor Necrosis Factor / genetics. Receptors, Tumor Necrosis Factor / physiology. Translocation, Genetic. Tumor Suppressor Protein p53 / physiology. bcl-2-Associated X Protein

  • MedlinePlus Health Information. consumer health - Autoimmune Diseases.
  • NCI CPTAC Assay Portal. NCI CPTAC Assay Portal .
  • NCI CPTAC Assay Portal. NCI CPTAC Assay Portal .
  • NCI CPTAC Assay Portal. NCI CPTAC Assay Portal .
  • NCI CPTC Antibody Characterization Program. NCI CPTC Antibody Characterization Program .
  • The Lens. Cited by Patents in .
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 11397650.001).
  • [ISSN] 0027-5107
  • [Journal-full-title] Mutation research
  • [ISO-abbreviation] Mutat. Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Adaptor Proteins, Signal Transducing; 0 / Antigens, CD95; 0 / BAX protein, human; 0 / BCL10 protein, human; 0 / Cysteine Proteinase Inhibitors; 0 / FASLG protein, human; 0 / Fas Ligand Protein; 0 / Membrane Glycoproteins; 0 / NAIP protein, human; 0 / Neoplasm Proteins; 0 / Nerve Tissue Proteins; 0 / Neuronal Apoptosis-Inhibitory Protein; 0 / Pore Forming Cytotoxic Proteins; 0 / Proto-Oncogene Proteins; 0 / Proto-Oncogene Proteins c-bcl-2; 0 / Receptors, Tumor Necrosis Factor; 0 / Tumor Suppressor Protein p53; 0 / bcl-2-Associated X Protein; 126465-35-8 / Perforin; EC 3.4.22.- / Caspases
  • [Number-of-references] 186
  •  go-up   go-down


11. Silvana B, Antonella LM, Basilia P, Trombetta D, Saija A, Salpietro C: Rituximab for the treatment of post-bone marrow transplantation refractory hemolytic anemia in a child with Omenn's syndrome. Pediatr Transplant; 2007 Aug;11(5):552-6
PDF icon [Fulltext service] Get downloadable fulltext PDFs of articles closely matching to this article, as many as you want.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Rituximab for the treatment of post-bone marrow transplantation refractory hemolytic anemia in a child with Omenn's syndrome.
  • Omenn's syndrome is a rare severe combined immunodeficiency that kills affected subjects before the end of the first year of life unless patients are treated with bone marrow transplantation (BMT).
  • Unfortunately, post-BMT patients may develop autoimmune diseases, such as autoimmune hemolytic anemia (AIHA), which sometimes fails to respond to standard therapies.
  • Rituximab is currently only labeled for treatment of B-cell lymphoproliferative disorders, such as B-cell non-Hodgkin's lymphoma and follicular lymphoma; however, it is also employed in the treatment of a variety of disorders mediated by auto-antibodies, such as AIHA and transplant-related autoimmune disorders.
  • Herein, we describe the case of a 23-month-old male child with Omenn's syndrome, who had undergone BMT and was successfully treated with rituximab (375 mg/m(2) intravenously, weekly for three times) for refractory post-BMT hemolytic anemia.
  • Our findings evidence that rituximab should be considered for treatment of post-BMT AIHA refractory to traditional therapy also in children with primary immunodeficiencies; furthermore, rituximab might represent a means to obtain remissions without the toxic effects associated with corticosteroid and immunosuppressive agents.
  • [MeSH-major] Anemia, Hemolytic / drug therapy. Anemia, Refractory / drug therapy. Antibodies, Monoclonal / therapeutic use. Bone Marrow Transplantation / adverse effects. Immunologic Factors / therapeutic use. Severe Combined Immunodeficiency / surgery
  • [MeSH-minor] Antibodies, Monoclonal, Murine-Derived. Antigens, CD20. Follow-Up Studies. Humans. Infant. Injections, Intravenous. Male. Postoperative Complications. Rituximab. Syndrome

  • Genetic Alliance. consumer health - CHILD Syndrome.
  • Genetic Alliance. consumer health - Anemia.
  • Genetic Alliance. consumer health - Transplantation.
  • MedlinePlus Health Information. consumer health - Bone Marrow Transplantation.
  • Hazardous Substances Data Bank. RITUXIMAB .
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 17631027.001).
  • [ISSN] 1397-3142
  • [Journal-full-title] Pediatric transplantation
  • [ISO-abbreviation] Pediatr Transplant
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Murine-Derived; 0 / Antigens, CD20; 0 / Immunologic Factors; 4F4X42SYQ6 / Rituximab
  •  go-up   go-down


12. Liebman H: Other immune thrombocytopenias. Semin Hematol; 2007 Oct;44(4 Suppl 5):S24-34
PDF icon [Fulltext service] Get downloadable fulltext PDFs of articles closely matching to this article, as many as you want.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Commonly occurring conditions associated with secondary ITP include lymphoproliferative disorders (chronic lymphocytic leukemia [CLL], Hodgkin's disease and non-Hodgkin's lymphomas), autoimmune collagen vascular diseases (systemic lupus erythematosus [SLE], thyroid disease, antiphospholipid syndrome [APS]), and chronic infections (human immunodeficiency virus [HIV], Helicobacter pylori, hepatitis C virus [HCV]).
  • The mechanism of platelet destruction in thrombocytopenias associated with lymphoproliferative disorders and collagen vascular diseases is identical to the autoimmune mechanism seen in primary ITP.
  • Drug-induced thrombocytopenias are uncommon and generally resolve quickly upon drug discontinuation, but are often attributed to other causes.
  • Platelet destruction in infection-associated ITP occurs via various mechanisms including accelerated platelet clearance due to immune complex disease as seen in HIV infection or cross-reactivity of anti-platelet glycoprotein antibodies and viral antigens in HIV, HCV, and H pylori infections (antigenic mimicry).
  • [MeSH-minor] Acquired Immunodeficiency Syndrome / complications. Acquired Immunodeficiency Syndrome / drug therapy. Acquired Immunodeficiency Syndrome / immunology. Antiphospholipid Syndrome / complications. Antiphospholipid Syndrome / drug therapy. Antiphospholipid Syndrome / immunology. Diagnosis, Differential. Female. Helicobacter Infections / complications. Helicobacter Infections / drug therapy. Helicobacter Infections / immunology. Hepatitis C / complications. Hepatitis C / drug therapy. Hepatitis C / immunology. Humans. Leukemia / complications. Leukemia / drug therapy. Leukemia / immunology. Lupus Erythematosus, Systemic / complications. Lupus Erythematosus, Systemic / drug therapy. Lupus Erythematosus, Systemic / immunology. Lymphoma / complications. Lymphoma / immunology. Lymphoma / therapy. Male. Platelet Count. Thyroid Diseases / complications. Thyroid Diseases / immunology. Thyroid Diseases / therapy


13. Quartuccio L, De Re V, Fabris M, Marzotto A, Franzolini N, Gasparotto D, Caggiari L, Ferraccioli G, Scott CA, De Vita S: Atypical lymphoproliferation progressing into B-cell lymphoma in rheumatoid arthritis treated with different biological agents: clinical course and molecular characterization. Haematologica; 2006 May;91(5):691-4
PDF icon [Fulltext service] Download fulltext PDF of this article and others, as many as you want.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Atypical lymphoproliferation progressing into B-cell lymphoma in rheumatoid arthritis treated with different biological agents: clinical course and molecular characterization.
  • A patient with rheumatoid arthritis (RA) developed an atypical lymphoproliferative disorder (LPD) after methotrexate and cyclosporine A, which regressed after suspension of both drugs.
  • Anti-tumor necrosis factor a therapy was used when the arthritis relapsed, but an aggressive B-cell non Hodgkin's lymphoma developed.
  • A minor clone with significant sequence homology to B-cell lymphomas arising in Sjogren's syndrome and mixed cryoglobulinemia syndrome, given rise to the non-Hodgkin's lymphoma.
  • Treatment of rheumatoid arthritis associated with lymphoproliferation represents a clinical challenge, and common pathogenetic pathways to lymphoma may occur in different autoimmune diseases.
  • [MeSH-major] Antirheumatic Agents / therapeutic use. Arthritis, Rheumatoid / complications. Immunosuppressive Agents / therapeutic use. Lymphoma, Large B-Cell, Diffuse / etiology. Lymphoproliferative Disorders / complications
  • [MeSH-minor] Aged. Antibodies, Monoclonal / therapeutic use. Antibodies, Monoclonal, Murine-Derived. Autoimmune Diseases / complications. Cyclosporine / adverse effects. Cyclosporine / therapeutic use. DNA, Neoplasm / genetics. Disease Progression. Disease Susceptibility. Etanercept. Female. Gene Rearrangement, B-Lymphocyte, Heavy Chain. Gene Rearrangement, B-Lymphocyte, Light Chain. Genes, Immunoglobulin. Humans. Immunocompromised Host. Immunoglobulin G / adverse effects. Immunoglobulin G / contraindications. Immunoglobulin G / therapeutic use. Methotrexate / adverse effects. Methotrexate / therapeutic use. Neoplasm Proteins / analysis. Neoplasm Proteins / genetics. Receptors, Tumor Necrosis Factor / therapeutic use. Rituximab. Tumor Necrosis Factor-alpha / antagonists & inhibitors

  • Genetic Alliance. consumer health - Arthritis.
  • Genetic Alliance. consumer health - Rheumatoid arthritis.
  • MedlinePlus Health Information. consumer health - Rheumatoid Arthritis.
  • Hazardous Substances Data Bank. RITUXIMAB .
  • Hazardous Substances Data Bank. Etanercept .
  • Hazardous Substances Data Bank. METHOTREXATE .
  • Hazardous Substances Data Bank. CYCLOSPORIN A .
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 16670074.001).
  • [ISSN] 1592-8721
  • [Journal-full-title] Haematologica
  • [ISO-abbreviation] Haematologica
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Murine-Derived; 0 / Antirheumatic Agents; 0 / DNA, Neoplasm; 0 / Immunoglobulin G; 0 / Immunosuppressive Agents; 0 / Neoplasm Proteins; 0 / Receptors, Tumor Necrosis Factor; 0 / Tumor Necrosis Factor-alpha; 4F4X42SYQ6 / Rituximab; 83HN0GTJ6D / Cyclosporine; OP401G7OJC / Etanercept; YL5FZ2Y5U1 / Methotrexate
  •  go-up   go-down


14. Papiris SA, Kalomenidis I, Malagari K, Kapotsis GE, Harhalakis N, Manali ED, Rontogianni D, Roussos C, Moutsopoulos HM: Extranodal marginal zone B-cell lymphoma of the lung in Sjögren's syndrome patients: reappraisal of clinical, radiological, and pathology findings. Respir Med; 2007 Jan;101(1):84-92
PDF icon [Fulltext service] Download fulltext PDF of this article and others, as many as you want.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Extranodal marginal zone B-cell lymphoma of the lung in Sjögren's syndrome patients: reappraisal of clinical, radiological, and pathology findings.
  • BACKGROUND: Primary Sjögren's syndrome (pSs) is an autoimmune rheumatic disease that may express in a small number of patients a spectrum of lymphoproliferative diseases.
  • The aim of this study was to describe clinical, imaging and pathology features of the extranodal marginal zone B-cell lymphoma (MZCL) of the lung of mucosa-associated lymphoid tissue (MALT) type in patients with pSs.
  • METHODS: All patients (N=10) with biopsy proven MZCL of the lung of MALT type diagnosed in a tertiary teaching hospital during the last 7 years were studied.
  • CONCLUSIONS: Lung MZCL associated with pSs are characterized by an important dissociation between clinical expression and radiological pattern.
  • Various chemotherapeutic agents in combination with rituximab lead to partial or complete remission in the majority of patients.
  • [MeSH-major] Lymphoma, B-Cell / complications. Lymphoma, B-Cell, Marginal Zone / complications
  • [MeSH-minor] B-Lymphocytes / pathology. Biomarkers / analysis. CD4-Positive T-Lymphocytes / pathology. Female. Humans. Immunohistochemistry / methods. Keratins / analysis. Lung / pathology. Lung / radiography. Male. Sjogren's Syndrome / complications. Sjogren's Syndrome / pathology. Sjogren's Syndrome / radiography. Tomography, X-Ray Computed

  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 16735112.001).
  • [ISSN] 0954-6111
  • [Journal-full-title] Respiratory medicine
  • [ISO-abbreviation] Respir Med
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers; 68238-35-7 / Keratins
  •  go-up   go-down


15. Rao VK, Dowdell KC, Dale JK, Dugan F, Pesnicak L, Bi LL, Hoffmann V, Penzak S, Avila NA, Fleisher TA, Puck JM, Straus SE: Pyrimethamine treatment does not ameliorate lymphoproliferation or autoimmune disease in MRL/lpr-/- mice or in patients with autoimmune lymphoproliferative syndrome. Am J Hematol; 2007 Dec;82(12):1049-55
PDF icon [Fulltext service] Download fulltext PDF of this article and others, as many as you want.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pyrimethamine treatment does not ameliorate lymphoproliferation or autoimmune disease in MRL/lpr-/- mice or in patients with autoimmune lymphoproliferative syndrome.
  • Autoimmune lymphoproliferative syndrome (ALPS) is an inherited disorder of lymphocyte apoptosis leading to childhood onset of marked lymphadenopathy, hepatosplenomegaly, autoimmune cytopenias, and increased risk of lymphoma.
  • Most cases are associated with heterozygous mutations in the gene encoding Fas protein.
  • Prolonged use of immunosuppressive drugs that do ameliorate its autoimmune complications fail to consistently lessen lymphoproliferation in ALPS.
  • A case series had described children with ALPS, whose spleens (SPL) and lymph nodes decreased in size when treated weekly with pyrimethamine and sulfadoxine; parallel in vitro studies showed only pyrimethamine to promote apoptosis.
  • Moreover, seven children with ALPS enrolled in a study of escalating dose of pyrimethamine alone given twice weekly for 12 weeks to determine if their lymphadenopathy and/or splenomegaly would diminish, as assessed by standardized computerized tomography.
  • Neither pyrimethamine alone or with sulfadoxine in the MRL/lpr-/- mice, nor pyrimethamine alone in ALPS patients proved efficacious.
  • We conclude that these drugs do not warrant further use empirically or as part of clinical trials in ALPS Type Ia as a lympholytic agent.
  • [MeSH-major] Autoimmune Diseases / drug therapy. Lymphoproliferative Disorders / drug therapy. Lymphoproliferative Disorders / immunology. Pyrimethamine / therapeutic use
  • [MeSH-minor] Animals. Apoptosis. Cell Death / drug effects. Child. Humans. Infant. Infant, Newborn. Lymph Nodes / immunology. Lymph Nodes / pathology. Mice. Mice, Inbred MRL lpr. Mice, Knockout. Spleen / immunology. Spleen / pathology. Treatment Failure

  • Genetic Alliance. consumer health - Autoimmune Lymphoproliferative Syndrome.
  • MedlinePlus Health Information. consumer health - Autoimmune Diseases.
  • COS Scholar Universe. author profiles.
  • Hazardous Substances Data Bank. Pyrimethamine .
  • NCI CPTAC Assay Portal. NCI CPTAC Assay Portal .
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 17674358.001).
  • [ISSN] 0361-8609
  • [Journal-full-title] American journal of hematology
  • [ISO-abbreviation] Am. J. Hematol.
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / /
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural
  • [Publication-country] United States
  • [Chemical-registry-number] Z3614QOX8W / Pyrimethamine
  •  go-up   go-down


16. Tilakaratne W, Dissanayake M: Paraneoplastic pemphigus: a case report and review of literature. Oral Dis; 2005 Sep;11(5):326-9
PDF icon [Fulltext service] Get downloadable fulltext PDFs of articles closely matching to this article, as many as you want.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease frequently associated with lymphoproliferative disorders.
  • Most patients develop very severe oral ulceration and conjunctival ulceration with or without genital ulceration resembling the features of Steven's Johnson's syndrome or most severe forms of drug eruptions.
  • We document a case of PNP in a 29-year-old female who suffers from non-Hodgkin's lymphoma.
  • [MeSH-major] Lymphoma, Non-Hodgkin / complications. Oral Ulcer / etiology. Paraneoplastic Syndromes / pathology. Pemphigus / pathology
  • [MeSH-minor] Adult. Anti-Inflammatory Agents / therapeutic use. Conjunctivitis / etiology. Female. Fluorescent Antibody Technique, Direct. Humans

  • Genetic Alliance. consumer health - Pemphigus.
  • MedlinePlus Health Information. consumer health - Pemphigus.
  • NCI CPTAC Assay Portal. NCI CPTAC Assay Portal .
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 16120122.001).
  • [ISSN] 1354-523X
  • [Journal-full-title] Oral diseases
  • [ISO-abbreviation] Oral Dis
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Anti-Inflammatory Agents
  •  go-up   go-down


17. Ramos-Casals M, Font J: Extrahepatic manifestations in patients with chronic hepatitis C virus infection. Curr Opin Rheumatol; 2005 Jul;17(4):447-55
PDF icon [Fulltext service] Get downloadable fulltext PDFs of articles closely matching to this article, as many as you want.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • PURPOSE OF REVIEW: Chronic hepatitis C virus infection often has autoimmune clinical and analytic features.
  • This review analyzes recent data on the close association of chronic hepatitis C virus infection with autoimmune and lymphoproliferative processes.
  • RECENT FINDINGS: Hepatitis C virus infection has been associated with both organ-specific (thyroiditis, diabetes) and systemic autoimmune diseases.
  • Experimental, virologic, and clinical evidence has demonstrated a close association between hepatitis C virus infection and Sjögren syndrome, with hepatitis C virus-associated Sjögren syndrome being indistinguishable in most cases from the primary form.
  • Hepatitis C virus has also been associated with an atypical presentation of antiphospholipid syndrome, as well as with the development of sarcoidosis.
  • A higher prevalence of hematologic processes in patients with hepatitis C virus infection has recently been reported, including cytopenias and lymphoproliferative disorders.
  • Recent data are available on the use of new immunosuppressive and biologic agents (mainly mycophenolate mofetil, anti-tumor necrosis factor agents, and rituximab) in patients with hepatitis C virus infection and autoimmune or lymphoproliferative manifestations.
  • SUMMARY: There is increasing evidence of a close association of hepatitis C virus infection with autoimmune and hematologic processes.
  • The sialotropism of hepatitis C virus may explain the close association with Sjögren syndrome, and its lymphotropism links the virus to cryoglobulinemia, autoimmune cytopenias, and lymphoma.
  • The substantial overlap between cryoglobulinemic features and the classification criteria for some systemic autoimmune diseases (systemic lupus erythematosus, rheumatoid arthritis, and polyarteritis nodosa) make the differentiation between mimicking and coexistence difficult.
  • [MeSH-major] Autoimmune Diseases / complications. Hepatitis C, Chronic / complications. Lymphoproliferative Disorders / complications

  • Genetic Alliance. consumer health - Hepatitis.
  • MedlinePlus Health Information. consumer health - Autoimmune Diseases.
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 15956842.001).
  • [ISSN] 1040-8711
  • [Journal-full-title] Current opinion in rheumatology
  • [ISO-abbreviation] Curr Opin Rheumatol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Number-of-references] 111
  •  go-up   go-down


18. Vezzoli P, Berti E, Marzano AV: Rationale and efficacy for the use of rituximab in paraneoplastic pemphigus. Expert Rev Clin Immunol; 2008 May;4(3):351-63
PDF icon [Fulltext service] Get downloadable fulltext PDFs of articles closely matching to this article, as many as you want.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Paraneoplastic pemphigus (PNP) is a life-threatening, autoimmune, blistering-skin disease, associated with various neoplasms, particularly lymphoproliferative disorders.
  • To define this condition, the encompassing term 'paraneoplastic autoimmune multiorgan syndrome' has also been suggested.
  • The anti-CD20 monoclonal antibody, rituximab, was successfully administered to two patients with PNP and CD20(+) follicular lymphoma in 2001.
  • Since then, good responses to rituximab by different refractory autoimmune disorders have been reported, but further controlled trials are warranted to evaluate the effectiveness and safety of this agent as a second-line treatment for PNP.

  • NCI CPTAC Assay Portal. NCI CPTAC Assay Portal .
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 20476925.001).
  • [ISSN] 1744-8409
  • [Journal-full-title] Expert review of clinical immunology
  • [ISO-abbreviation] Expert Rev Clin Immunol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  •  go-up   go-down


19. Straus SE, Jaffe ES, Puck JM, Dale JK, Elkon KB, Rösen-Wolff A, Peters AM, Sneller MC, Hallahan CW, Wang J, Fischer RE, Jackson CE, Lin AY, Bäumler C, Siegert E, Marx A, Vaishnaw AK, Grodzicky T, Fleisher TA, Lenardo MJ: The development of lymphomas in families with autoimmune lymphoproliferative syndrome with germline Fas mutations and defective lymphocyte apoptosis. Blood; 2001 07 01;98(1):194-200
PDF icon [Fulltext service] Download fulltext PDF of this article and others, as many as you want.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The development of lymphomas in families with autoimmune lymphoproliferative syndrome with germline Fas mutations and defective lymphocyte apoptosis.
  • Lymphomas were studied in kindreds with autoimmune lymphoproliferative syndrome (ALPS; Canale-Smith syndrome), a disorder of lymphocyte homeostasis usually associated with germline Fas mutations.
  • Lymphoma phenotype was determined by immunohistochemistry, frequency of CD3(+)CD4(-)CD8(-) T-cell-receptor alpha/beta cells by flow cytometry, nucleotide sequences of the gene encoding Fas (APT1, TNFRSF6), and the percentage of lymphocytes undergoing apoptosis in vitro.
  • Investigation of these 10 patients and their relatives with Fas mutations revealed that all had defective lymphocyte apoptosis and most had other features of ALPS.
  • [MeSH-major] Antigens, CD95 / genetics. Autoimmune Diseases / complications. Lymphoma / etiology. Lymphoproliferative Disorders / complications
  • [MeSH-minor] Adult. Apoptosis / drug effects. Apoptosis / genetics. Child. Family Health. Female. Germ-Line Mutation. Humans. Lymphocytes / pathology. Male. Middle Aged. Syndrome


20. De Sanctis LB, Mandreoli M, Poggi C, Pileri S, Casanova S, Santoro A: Acute renal failure in a young woman with Fisher-Evans' syndrome. J Nephrol; 2004 Sep-Oct;17(5):739-43
PDF icon [Fulltext service] Get downloadable fulltext PDFs of articles closely matching to this article, as many as you want.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Acute renal failure in a young woman with Fisher-Evans' syndrome.
  • A young female with Fisher-Evans' syndrome and a previous melanoma developed acute renal failure with generalized lymphoadenopathy and fever.
  • The appearance of renal lesions is common in the course of several hematological disorders, but is unusual in Fisher-Evans' syndrome.
  • Fisher-Evans' syndrome, defined as Coombs' positive hemolytic anemia and immune thrombocytopenia, is more frequently associated with the other autoimmune diseases, but not with renal involvement.
  • In our case report, having excluded amyloidosis, myeloma, interstitial nephritis and sepsis, the rapid involvement of renal function with enlarged renal size seemed to suggest renal lymphoma.
  • Polyclonal lymphocyte infiltration in a patient with a persistent autoimmune disease made us suspect a hyperimmune reaction.
  • This syndrome is a non-neoplastic proliferation of B-cells involving an exaggeration of lymphocyte transformation.
  • However, the clinical course is progressive and fatal, and can trigger a lymphoproliferative systemic disease.
  • In our patient, two elements led us to suspect it was not a typical hyperimmune syndrome: first, polyclonal lymphocytes had massively infiltrated the kidney and, secondly, the clinical outcome was extremely favorable.
  • Infective or toxic agents or drugs such as cyprofloxacin could have triggered the phenomenon, in the presence of a favorable condition such as Fisher-Evans' syndrome.
  • [MeSH-minor] Adult. Female. Humans. Lymphatic Diseases / diagnosis. Lymphatic Diseases / etiology. Lymphatic Diseases / immunology. Syndrome

  • Genetic Alliance. consumer health - Evans Syndrome.
  • Genetic Alliance. consumer health - Young syndrome.
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 15593044.001).
  • [ISSN] 1121-8428
  • [Journal-full-title] Journal of nephrology
  • [ISO-abbreviation] J. Nephrol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
  •  go-up   go-down


21. Alcântara C, Gomes MJ, Ferreira C: Rituximab therapy in primary Sjögren's syndrome. Ann N Y Acad Sci; 2009 Sep;1173:701-5
PDF icon [Fulltext service] Get downloadable fulltext PDFs of articles closely matching to this article, as many as you want.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Rituximab therapy in primary Sjögren's syndrome.
  • Primary Sjögren's syndrome is an autoimmune disease characterized by lymphocytic infiltration of glandular tissues leading to sicca symptoms, namely, dry eyes and dry mouth.
  • A small but not insignificant percentage of those patients evolve to B cell lymphoma.
  • The increased expression of B cell survival factors, such as B cell activating factor, may promote the perpetuation of a B cell clone and precede the lymphoproliferative disease.
  • Rituximab, a chimeric monoclonal antibody to CD20, leads to B cell depletion and may have a role in Sjögren systemic manifestations as well as in preventing and treating Sjögren-associated lymphoma.
  • [MeSH-major] Antibodies, Monoclonal / therapeutic use. Antigens, CD20 / immunology. Sjogren's Syndrome / drug therapy
  • [MeSH-minor] Antibodies, Monoclonal, Murine-Derived. Antirheumatic Agents / therapeutic use. B-Cell Activating Factor / metabolism. B-Lymphocytes / drug effects. B-Lymphocytes / immunology. B-Lymphocytes / metabolism. Humans. Rituximab. Treatment Outcome

  • MedlinePlus Health Information. consumer health - Sjogren's Syndrome.
  • Hazardous Substances Data Bank. RITUXIMAB .
  • [Email] Email this result item
    Email the results to the following email address:   [X] Close
  • (PMID = 19758218.001).
  • [ISSN] 1749-6632
  • [Journal-full-title] Annals of the New York Academy of Sciences
  • [ISO-abbreviation] Ann. N. Y. Acad. Sci.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Murine-Derived; 0 / Antigens, CD20; 0 / Antirheumatic Agents; 0 / B-Cell Activating Factor; 4F4X42SYQ6 / Rituximab
  •  go-up   go-down






Advertisement