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1. Zhong LY, Liu TH, Li YQ, Geng SX, Lu ZS, Weng JY, Wu SJ, Luo CW, Du X: [Serum proteomics in patients with RAEB myelodysplastic syndromes]. Nan Fang Yi Ke Da Xue Xue Bao; 2009 Sep;29(9):1799-801
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  • [Title] [Serum proteomics in patients with RAEB myelodysplastic syndromes].
  • OBJECTIVE: To screen the molecular markers for refractory anemia with excess blasts in transformation (RAEB) in myelodysplastic syndromes (MDS) by serum proteome profiling.
  • METHODS: The serum protein were isolated from patients with RAEB, acute myeloid leukemia or normal subjects by 2-dimensional electrophoresis (2-DE), and the electrophoresis gels were obtained to identify the differentially reacting protein spots.
  • RESULTS: Seven differentially expressed proteins in RAEB were found by 2-DE.
  • Of the 7 proteins, 4 were identified by MALDI-TOF-MS to have significantly differential expression in RAEB, including dipeptidyl peptidase (DPP/CD26), polymerase (DNA directed) kappa, PRO2044 and an albumin-like protein.
  • DDP/CD26 has increased expression in the serum in RAEB subtype MDS, suggesting its possible role in advanced MDS.
  • [MeSH-major] Anemia, Refractory, with Excess of Blasts / blood. DNA-Directed DNA Polymerase / blood. Dipeptidyl-Peptidases and Tripeptidyl-Peptidases / blood. Myelodysplastic Syndromes / blood. Proteomics

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  • (PMID = 19778794.001).
  • [ISSN] 1673-4254
  • [Journal-full-title] Nan fang yi ke da xue xue bao = Journal of Southern Medical University
  • [ISO-abbreviation] Nan Fang Yi Ke Da Xue Xue Bao
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] China
  • [Chemical-registry-number] EC 2.7.7.7 / DNA-Directed DNA Polymerase; EC 2.7.7.7 / POLK protein, human; EC 3.4.14.- / Dipeptidyl-Peptidases and Tripeptidyl-Peptidases
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2. Takizawa A, Miura T, Fujinami K, Osada Y, Tanaka M, Maruta I: [A case of therapy-related leukemia/myelodysplastic syndrome following treatment of refractory testicular germ cell tumor]. Nihon Hinyokika Gakkai Zasshi; 2005 Nov;96(7):701-4
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  • [Title] [A case of therapy-related leukemia/myelodysplastic syndrome following treatment of refractory testicular germ cell tumor].
  • We report a patient with a refractory testicular non-seminomatous germ cell tumor (NSGCT) who developed therapy-related leukemia (TRL) after undergoing salvage chemotherapy and multiple operations for repeat recurrences.
  • A bone marrow examination showed evidence of myelodysplastic syndrome (MDS) and refractory anemia with excess of blasts in transformation (RAEB in T) under French-America-British (FAB) classification.
  • Since early detection and treatment are necessary for the management of TRL, peripheral blood examinations should be performed after a diagnosis of refractory germ cell tumor has been made.
  • [MeSH-minor] Adult. Anemia, Refractory, with Excess of Blasts / pathology. Antineoplastic Combined Chemotherapy Protocols / adverse effects. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cisplatin / administration & dosage. Cisplatin / adverse effects. Drug Administration Schedule. Etoposide / administration & dosage. Etoposide / adverse effects. Humans. Ifosfamide / administration & dosage. Ifosfamide / adverse effects. Male


3. Giraldo P, Nomdedeu B, Loscertales J, Requena C, de Paz R, Tormo M, Navarro P, Benedit P, Gasquet JA, Aranesp in Myelodysplastic Syndromes (ARM) Study Group: Darbepoetin alpha for the treatment of anemia in patients with myelodysplastic syndromes. Cancer; 2006 Dec 15;107(12):2807-16
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  • [Title] Darbepoetin alpha for the treatment of anemia in patients with myelodysplastic syndromes.
  • BACKGROUND: Anemia occurs as a comorbidity in from 80% to 85% of patients with myelodysplastic syndromes (MDS): It causes fatigue, increases transfusion needs, and reduces quality of life.
  • According to the French-American-British classification system (n = 81 patients), 39.5% had refractory anemia (RA), 46.9% had RA with ringed sideroblasts, 9.9% had RA with excess blasts (RAEB), 1.2% had RAEB in transformation, and 2.5% had chronic myelomonocytic leukemia.
  • [MeSH-major] Anemia / drug therapy. Erythropoietin / analogs & derivatives. Hematinics / therapeutic use. Myelodysplastic Syndromes / complications


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4. Scott BL, Storer B, Loken MR, Storb R, Appelbaum FR, Deeg HJ: Pretransplantation induction chemotherapy and posttransplantation relapse in patients with advanced myelodysplastic syndrome. Biol Blood Marrow Transplant; 2005 Jan;11(1):65-73
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  • However, treatment-related toxicity and, in patients with advanced MDS (refractory anemia with excess blasts [RAEB]; RAEB in transformation [RAEB-T]) or transformation to acute myeloid leukemia with multilineage dysplasia (tAML), posttransplantation relapse continue to be prevalent.
  • Thirty-three patients (3 with RAEB, 6 with RAEB-T, and 24 with tAML) received IC before transplantation, and 92 patients (62 with RAEB, 22 with RAEB-T, and 8 with tAML) did not.
  • There was no evidence of a benefit in posttransplantation outcome associated with prior IC, either for patients with RAEB/RAEB-T or those with tAML, with either conditioning regimen.
  • [MeSH-minor] Adolescent. Adult. Aged. Anemia, Refractory, with Excess of Blasts / therapy. Child. Child, Preschool. Female. Humans. Leukemia, Myeloid / therapy. Male. Middle Aged. Recurrence. Remission Induction / methods. Retrospective Studies. Severity of Illness Index. Survival Analysis. Treatment Outcome

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  • (PMID = 15625546.001).
  • [ISSN] 1083-8791
  • [Journal-full-title] Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation
  • [ISO-abbreviation] Biol. Blood Marrow Transplant.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA15704; United States / NCI NIH HHS / CA / CA18029; United States / NCI NIH HHS / CA / CA87948; United States / NHLBI NIH HHS / HL / HL 36444
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
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5. Santini V, Fenaux P, Mufti GJ, Hellström-Lindberg E, Silverman LR, List A, Gore SD, Seymour JF, Backstrom J, Beach CL: Management and supportive care measures for adverse events in patients with myelodysplastic syndromes treated with azacitidine*. Eur J Haematol; 2010 Aug;85(2):130-8
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  • In the AZA-001 study, patients with higher-risk MDS (FAB-defined refractory anemia with excess blasts (RAEB), RAEB in transformation, or chronic myelomonocitic leukaemia and IPSS int-2 or high) were randomized to azacitidine or to conventional care regimens (CCR), which included low-dose ara-C, BSC, or intensive chemotherapy.

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  • [Cites] J Clin Oncol. 2002 May 15;20(10):2429-40 [12011120.001]
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  • (PMID = 20394651.001).
  • [ISSN] 1600-0609
  • [Journal-full-title] European journal of haematology
  • [ISO-abbreviation] Eur. J. Haematol.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA 31946; United States / NCI NIH HHS / CA / U10 CA031946; United States / NCI NIH HHS / CA / K24 CA111717; United States / NCI NIH HHS / CA / U10 CA033601; United States / NCI NIH HHS / CA / CA 33601
  • [Publication-type] Clinical Trial, Phase III; Journal Article; Multicenter Study; Randomized Controlled Trial; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antimetabolites, Antineoplastic; M801H13NRU / Azacitidine
  • [Other-IDs] NLM/ NIHMS490297; NLM/ PMC4000014
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6. Inoue H, Matsushita K, Arima N, Hamada H, Uozumi K, Ozaki A, Akimoto M, Kawada H, Kukita T, Yoshimitsu M, Matsumoto T, Tei C: High prevalence of human T-lymphotropic virus type I carriers among patients with myelodysplastic syndrome refractory anemia with excess of blasts (RAEB), RAEB in transformation and acute promyelocytic leukemia. Leuk Lymphoma; 2008 Feb;49(2):315-21
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  • [Title] High prevalence of human T-lymphotropic virus type I carriers among patients with myelodysplastic syndrome refractory anemia with excess of blasts (RAEB), RAEB in transformation and acute promyelocytic leukemia.
  • We examined human T-lymphotropic virus type I (HTLV-I) infection among patients with myelodysplastic syndrome (MDS), refractory anemia with excess of blasts (RAEB)/RAEB in transformation (RAEBt) and acute myelogenous leukemia (AML).
  • The study population consisted of 151 patients: 46 with MDS RAEB/RAEBt and 105 with AML (M1, n = 15; M2, n = 39; M3, n = 18; M4, n = 19; M5, n = 9; M6, n = 3; M7, n = 2).
  • As a reference, we examined 92 patients with refractory anemia (RA) and 405 patients with cardiovascular diseases (CVD).
  • Thirteen patients with RAEB/RAEBt (28.3%), 11 with AML (11.6%), 27 with RA (29.3%), and 45 with CVD (11.0%) were positive for HTLV-I.
  • The prevalences of HTLV-I infection among patients with RAEB/RAEBt (P < 0.001), APL (P = 0.001), and RA (P < 0.001) were significantly higher than that in patients with CVD.
  • The prevalences of HTLV-I infection were still significantly higher in patients with RAEB/RAEBt (P = 0.007), APL (P = 0.017) and RA (P < 0.001) than in those with CVD matched by sex and age.
  • Platelet counts and survival times of RAEB/RAEBt patients with infection were significantly lower than those of patients without infection.
  • [MeSH-major] Anemia, Refractory, with Excess of Blasts / virology. HTLV-I Infections / epidemiology. Leukemia, Promyelocytic, Acute / virology. Myelodysplastic Syndromes / virology
  • [MeSH-minor] Adult. Aged. Cell Transformation, Neoplastic. Female. Humans. Male. Middle Aged. Platelet Count. Prevalence. Survival Rate


7. Breccia M, Latagliata R, Carmosino I, Gentilini F, D'Elia GM, Levi A, Natalino F, Frustaci A, De Cuia MR, Alimena G: Refractory anaemia with excess of blasts in transformation re-evaluated with the WHO criteria: identification of subgroups with different survival. Acta Haematol; 2007;117(4):221-5
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  • [Title] Refractory anaemia with excess of blasts in transformation re-evaluated with the WHO criteria: identification of subgroups with different survival.
  • One of the major changes suggested by the World Health Organization (WHO) classification with respect to the French-American-British (FAB) proposal for myelodysplastic syndromes (MDS) was to lower the bone marrow (BM) blast count from 30 to 20%, thus eliminating the refractory anaemia with excess of blasts in transformation (RAEB-t) category.
  • However, a general consensus has not been reached, and several authors still retain RAEB-t as an MDS sub-entity.
  • We re-evaluated our series of 74 patients classified as RAEB-t according to the FAB criteria by stratifying them into two subsets: patients with at least 5% peripheral blast (PB) cells but with BM blasts <20% (group I) and patients with BM blastosis between 20 and 30% and PBs <5% (group II).
  • We did not find differences as to the frequency of acute myeloid leukaemia transformation, but a significant difference was evidenced as to survival (9.3 vs. 16 months in group I vs. group II, respectively; p = 0.02).
  • Furthermore, at our institution, we compared the RAEB-t group I patients who, based on >5% PBs, should be included in the RAEB-II category according to the WHO criteria, with a group of 98 patients who were diagnosed as RAEB-II according to the WHO criteria.
  • The findings showed that the aggregation of these two subsets appeared inappropriate, because patients of the two groups showed different clinical features and rates of acute transformation.
  • In conclusion, the RAEB-t entity according to the FAB criteria, although including heterogeneous clinical patient subsets, should more likely be considered as an advanced stage of MDS, rather than a true acute myeloid leukaemia.
  • [MeSH-major] Anemia, Refractory, with Excess of Blasts / classification. Lymphocyte Activation. Survival Analysis

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  • [Copyright] 2007 S. Karger AG, Basel
  • (PMID = 17259693.001).
  • [ISSN] 1421-9662
  • [Journal-full-title] Acta haematologica
  • [ISO-abbreviation] Acta Haematol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
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8. Kaiafa G, Papadopoulos A, Ntaios G, Saouli Z, Savopoulos C, Tsesmeli N, Kontoninas Z, Chatzinikolaou A, Tsavdaridou V, Klonizakis I, Hatzitolios A: Detection of CD55- and CD59-deficient granulocytic populations in patients with myelodysplastic syndrome. Ann Hematol; 2008 Apr;87(4):257-62
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  • We studied 90 MDS patients: 19 patients with RA, 15 with refractory anemia with ringed sideroblasts (RARS), 18 with refractory anemia with excess of blasts (RAEB), 17 with refractory anemia with excess of blasts in transformation (RAEB-t), and 21 with chronic myelomonocytic leukemia (CMML).
  • [MeSH-major] Anemia, Refractory, with Excess of Blasts / blood. Antigens, CD55 / blood. Antigens, CD59 / blood. Granulocytes / immunology. Hemoglobinuria, Paroxysmal / blood. Myelodysplastic Syndromes / blood


9. Timeus F, Crescenzio N, Doria A, Foglia L, Linari A, Giaccone M, Pastore G, di Montezemolo LC, Ramenghi U, Saracco P: Flow cytometric evaluation of circulating CD34+ cell counts and apoptotic rate in children with acquired aplastic anemia and myelodysplasia. Exp Hematol; 2005 May;33(5):597-604
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  • [Title] Flow cytometric evaluation of circulating CD34+ cell counts and apoptotic rate in children with acquired aplastic anemia and myelodysplasia.
  • OBJECTIVE: Identification of a rapid and noninvasive test for the follow-up of aplastic anemia (AA) patients during immunosuppressive therapy (IST) to evaluate its functional effect on hematopoietic progenitors (HPC) and for early detection of progression to myelodysplasia or relapse.
  • MATERIALS AND METHODS: Absolute count and apoptotic rate (AR) of peripheral blood (PB) CD34+ cells were evaluated by three-color flow cytometry for CD45, CD34, and annexin V in cord blood (CB), normal children, and adults, as well as in pediatric patients with AA at diagnosis and during IST, Fanconi anemia (FA), chronic immune cytopenia, and refractory anemia with excess blasts (RAEB).
  • Conversely, in RAEB the AR was 11.7% +/- 3.5% and the absolute count 85.1 +/- 48.2/microL (p < 0.05).
  • CONCLUSIONS: Flow cytometry evaluation of PB CD34+ AR and counts is a noninvasive and feasible first-step method for the differentiation of AA and myelodysplasia (MDS), and it might be useful for monitoring AA during IST to secure the early detection of relapse or transformation to MDS.
  • [MeSH-major] Anemia, Aplastic / pathology. Antigens, CD34 / analysis. Apoptosis. Lymphocytes / immunology. Myelodysplastic Syndromes / pathology


10. Raza A, Lisak L, Billmeier J, Pervaiz H, Mumtaz M, Gohar S, Wahid K, Galili N: Phase II study of topotecan and thalidomide in patients with high-risk myelodysplastic syndromes. Leuk Lymphoma; 2006 Mar;47(3):433-40
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  • This phase II trial investigated the safety and preliminary efficacy of a topotecan/thalidomide combination therapy in patients with myelodysplastic syndrome who had refractory anemia with excess blasts (RAEB), RAEB with transformation, or chronic myelomonocytic anemia.
  • Approximately one-third of the patients had decreases in bone marrow blasts of 50%.
  • [MeSH-minor] Aged. Anemia, Refractory, with Excess of Blasts / drug therapy. Anemia, Refractory, with Excess of Blasts / pathology. Dose-Response Relationship, Drug. Drug Administration Schedule. Drug Therapy, Combination. Drug Tolerance. Female. Humans. Male. Middle Aged. Risk Factors. Time Factors. Treatment Outcome

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  • (PMID = 16396766.001).
  • [ISSN] 1042-8194
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase II; Journal Article; Randomized Controlled Trial; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 4Z8R6ORS6L / Thalidomide; 7M7YKX2N15 / Topotecan
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11. Wimazal F, Krauth MT, Vales A, Böhm A, Agis H, Sonneck K, Aichberger KJ, Mayerhofer M, Simonitsch-Klupp I, Müllauer L, Sperr WR, Valent P: Immunohistochemical detection of vascular endothelial growth factor (VEGF) in the bone marrow in patients with myelodysplastic syndromes: correlation between VEGF expression and the FAB category. Leuk Lymphoma; 2006 Mar;47(3):451-60
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  • We examined the expression of VEGF in paraffin-embedded bone marrow sections obtained from normal donors (n = 5) and 46 patients with myelodysplastic syndromes [MDS, French-American-British (FAB)-type refractory anemia (RA), n = 10; refractory anemia with ringed sideroblasts (RARS), n = 10; refractory anemia with excess blasts (RAEB), n = 10; RAEB in transformation (RAEB-T), n = 8; chronic myelomonocytic leukemia (CMML), n = 8] by immunohistochemistry using an anti-VEGF antibody.
  • Higher levels of VEGF were found in patients with MDS, subtypes RAEB, RAEB-T and CMML, compared to patients with RA or RARS, or the normal bone marrow.
  • These differences were found to result from expression of VEGF in immature myeloid cells in RAEB, RAEB-T and CMML.
  • The microvessel density was also higher in patients with RAEB-T and CMML compared to RA and RARS or the normal bone marrow.
  • The amount of expressed VEGF is related to the percentage of immature myeloid cells (blasts and monocytic progenitors) and correlates with the FAB category.
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Anemia, Refractory, with Excess of Blasts / metabolism. Anemia, Refractory, with Excess of Blasts / pathology. Female. Humans. Immunohistochemistry. Male. Middle Aged. Neovascularization, Pathologic / metabolism. Neovascularization, Pathologic / pathology. Predictive Value of Tests. RNA, Messenger / genetics. RNA, Messenger / metabolism. Receptors, Vascular Endothelial Growth Factor / antagonists & inhibitors. Receptors, Vascular Endothelial Growth Factor / genetics. Receptors, Vascular Endothelial Growth Factor / metabolism

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  • [CommentIn] Leuk Lymphoma. 2006 Mar;47(3):377-8 [16396758.001]
  • (PMID = 16396768.001).
  • [ISSN] 1042-8194
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / RNA, Messenger; 0 / Vascular Endothelial Growth Factors; EC 2.7.10.1 / Receptors, Vascular Endothelial Growth Factor
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12. Lorenzo F, Nishii K, Monma F, Kuwagata S, Usui E, Shiku H: Mutational analysis of the KIT gene in myelodysplastic syndrome (MDS) and MDS-derived leukemia. Leuk Res; 2006 Oct;30(10):1235-9
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  • The progenitor cells of myelodysplastic syndrome (MDS) are thought to undergo a multistep process during their transformation into overt acute leukemia.
  • Mutation was detected in 2 of 15 (13.3%) patients with refractory anemia with excess blasts transformation (RAEB-T), in 1 of 15 (6.6%) patients with chronic myelomonocytic leukemia (CMML), and in 5 of 26 (19.2%) patients with MDS-AML.
  • However, no mutation was found in any of the nine patients with refractory anemia (RA) or the 10 patients with refractory anemia with excess blasts (RAEB).
  • [MeSH-minor] Adult. Aged. Anemia, Refractory, with Excess of Blasts / genetics. Codon / genetics. DNA Mutational Analysis. Disease Progression. Female. Humans. Japan. Karyotyping. Male. Middle Aged. Reverse Transcriptase Polymerase Chain Reaction


13. Parikh SH, Mendizabal A, Martin PL, Prasad VK, Szabolcs P, Driscoll TA, Kurtzberg J: Unrelated donor umbilical cord blood transplantation in pediatric myelodysplastic syndrome: a single-center experience. Biol Blood Marrow Transplant; 2009 Aug;15(8):948-55
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  • MDS stage was refractory anemia (RA) in 12, refractory anemia with excess blasts (RAEB) in 8, and refractory anemia with excess blasts in transformation (RAEB-T) in 3 patients; 18 (78%) patients had primary MDS.
  • [MeSH-minor] Adolescent. Adult. Anemia, Refractory, with Excess of Blasts. Chemoprevention / methods. Child. Female. Graft vs Host Disease / drug therapy. Graft vs Host Disease / prevention & control. Humans. Male. Middle Aged. Retrospective Studies. Survival Analysis. Tissue Donors. Treatment Outcome. Whole-Body Irradiation. Young Adult


14. McGrattan P, Logan A, Humphreys M, Bowers M: Jumping translocation in acute monocytic leukemia (M5b) with alternative breakpoint sites in the long arm of donor chromosome 3. Med Oncol; 2010 Sep;27(3):667-72

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  • An 86-year-old man presented with acute hepatic failure, worsening thrombocytopenia, and anemia having been diagnosed and managed expectantly with cytogenetically normal RAEB-1.
  • After 20 months a diagnosis of disease transformation to acute monocytic leukemia (M5b) was made.
  • Conventional G-banded analysis of unstimulated peripheral blood cultures detected the proximal 3q1?2 JT clone involving recipient chromosome 10 several weeks after transformation to acute monocytic leukemia.
  • Palliative care was administered until his demise 2.2 months after disease transformation.
  • [MeSH-minor] Aged, 80 and over. Anemia, Refractory, with Excess of Blasts / genetics. Disease Progression. Humans. Male

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  • (PMID = 19629764.001).
  • [ISSN] 1559-131X
  • [Journal-full-title] Medical oncology (Northwood, London, England)
  • [ISO-abbreviation] Med. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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15. Jiang Y, Dunbar A, Gondek LP, Mohan S, Rataul M, O'Keefe C, Sekeres M, Saunthararajah Y, Maciejewski JP: Aberrant DNA methylation is a dominant mechanism in MDS progression to AML. Blood; 2009 Feb 5;113(6):1315-25
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  • Aberrant methylation was seen in every sample, on average affecting 91 of 1505 CpG loci in early MDS and 179 of 1505 loci after blast transformation (refractory anemia with excess blasts [RAEB]/AML).
  • In contrast, chromosome aberrations were seen in 79% of early MDS samples and 90% of RAEB/AML samples, and were not as widely distributed over the genome.

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  • (PMID = 18832655.001).
  • [ISSN] 1528-0020
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] ENG
  • [Grant] United States / NHLBI NIH HHS / HL / K24 HL077522; United States / NHLBI NIH HHS / HL / R01 HL082983; United States / NCRR NIH HHS / RR / S10 RR019391; United States / NCRR NIH HHS / RR / U54 RR019391
  • [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 / FZD3 protein, human; 0 / Frizzled Receptors; 0 / RNA, Messenger; 0 / Receptors, G-Protein-Coupled; 0 / Tumor Suppressor Proteins
  • [Other-IDs] NLM/ PMC2637194
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16. Yao DM, Qian J, Xu WR, Lin J, Jiang YW, Fei X, Han LX, Wang Y, Cen JN, Chen ZX: [Alteration of methylation status of fragile histidine triad gene promoter in patients with myelodysplastic syndrome]. Zhonghua Yi Xue Yi Chuan Xue Za Zhi; 2008 Feb;25(1):36-9
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  • Although significant difference was not observed in the frequencies of FHIT gene hypermethylation among patients with refractory anemia/refractory anemia with ringed sideroblasts (RA/RAS) (1/6, 16.7%), refractory anemia/refractory anemia with ringed sideroblasts (RCMD) and refractory cytopenia with multilineage dysplasia with ringed blasts (RCMD-RS) (6/19, 31.6%), refractory anemia with excess blasts-1 (RAEB-1) (7/11, 63.6%), refractory anemia with excess blasts-2 (RAEB-2) (4/7, 57.1%) and refractory anemia with excess blasts in transformation/acute myeloid leukemia (RAEBt/AML) (8/11, 72.7%)(chi-square=8.417, P=0.077), it was observed in patients in early stages (RA/RAS and RCMD) (7/25, 28.0%), advanced stages (RAEB-1 and RAEB-2)(11/18, 61.1%) and RAEBt/AML (8/11, 72.7%) (chi-square=7.938, P=0.019).

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  • (PMID = 18247301.001).
  • [ISSN] 1003-9406
  • [Journal-full-title] Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics
  • [ISO-abbreviation] Zhonghua Yi Xue Yi Chuan Xue Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Neoplasm Proteins; 0 / fragile histidine triad protein; EC 3.6.- / Acid Anhydride Hydrolases
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17. Qi H, Xiao L, Lingyun W, Ying T, Yi-Zhi L, Shao-Xu Y, Quan P: Expression of type 1 insulin-like growth factor receptor in marrow nucleated cells in malignant hematological disorders: correlation with apoptosis. Ann Hematol; 2006 Feb;85(2):95-101
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  • In MDS nucleated cells, IGF-IR showed stronger expression in refractory anemia with excess blasts (RAEB)/RAEB in transformation/chronic myelomonocytic leukemia subgroup when compared to RA/RA with ringed sideroblasts cases (64.1+/-3.2 vs 53.5+/-16.2%) (P>0.05).
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Anemia / metabolism. Cell Transformation, Neoplastic. Child. Female. Humans. Male. Middle Aged


18. Bacher U, Haferlach T, Kern W, Haferlach C, Schnittger S: A comparative study of molecular mutations in 381 patients with myelodysplastic syndrome and in 4130 patients with acute myeloid leukemia. Haematologica; 2007 Jun;92(6):744-52
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  • BACKGROUND AND OBJECTIVES: The precise relationship between myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML) is unclear and the role of molecular mutations in leukemic transformation in MDS is controversial.
  • DESIGN AND METHODS: We compared the frequency of FLT3-length mutations (FLT3-LM), FLT3-TKD, MLL-partial tandem duplications (MLL-PTD), NRAS, and KITD816 in 381 patients with MDS refractory anemia with excess blasts [RAEB] n=49; with ringed sideroblasts [RARS] n=310; chronic monomyelocytic leukemia [CMML] n=22) and in 4130 patients with AML (de novo: n=3139; secondary AML [s-AML] following MDS: n=397; therapy-related [t-AML]: n=233; relapsed: n=361).
  • RESULTS: All mutations were more frequent in s-AML than in MDS and all but the FLT3-TKD were more frequent in RAEB than in RA/RARS.
  • The higher incidences in s-AML were significant for FLT3-TKD (p=0.032), MLL-PTD (p=0.034), and FLT3-LM (RA/RARS: 0/45; RAEB: 8/293; 2.7%; s-AML: 45/389; 11.6%; p<0.0001).
  • FLT3-LM-acquisition occurred in 3/22 cases (13.6%) during MDS transformation; NRAS-acquisition occurred in 1/24 (4.2%).

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  • [CommentIn] Haematologica. 2007 Jun;92(6):723-7 [17550842.001]
  • (PMID = 17550846.001).
  • [ISSN] 1592-8721
  • [Journal-full-title] Haematologica
  • [ISO-abbreviation] Haematologica
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / MLL protein, human; 149025-06-9 / Myeloid-Lymphoid Leukemia Protein; EC 2.1.1.43 / Histone-Lysine N-Methyltransferase; EC 2.7.10.1 / FLT3 protein, human; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit; EC 2.7.10.1 / fms-Like Tyrosine Kinase 3; EC 3.6.5.2 / ras Proteins
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19. Harada H, Harada Y: Point mutations in the AML1/RUNX1 gene associated with myelodysplastic syndrome. Crit Rev Eukaryot Gene Expr; 2005;15(3):183-96
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  • Recently, we reported the high incidence of somatic mutations in the AML1/RUNX1 gene (which is a critical regulator of definitive hematopoiesis and the most frequent target for translocation of acute myeloid leukemia [AML]) in MDS, especially refractory anemia with excess blasts (RAEB), RAEB in transformation (RAEBt), and AML following MDS (defined here as MDS/AML).

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  • (PMID = 16390315.001).
  • [ISSN] 1045-4403
  • [Journal-full-title] Critical reviews in eukaryotic gene expression
  • [ISO-abbreviation] Crit. Rev. Eukaryot. Gene Expr.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Core Binding Factor Alpha 2 Subunit; 0 / RUNX1 protein, human
  • [Number-of-references] 78
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20. Xu LP, Huang XJ, Liu KY, Chen H, Liu DH, Han W, Chen YH, Gao ZY, Lu J, Wang JZ, Lu DP: [Allogenic stem cell transplantation from genotypically HLA-identical siblings for 30 patients with myelodysplastic syndromes]. Zhonghua Xue Ye Xue Za Zhi; 2006 Aug;27(8):518-21
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  • On HSCT, 4 patients had refractory anemia (RA) , 2 RA with ringed sideroblasts (RARS) , 7 RA with excess blasts(RAEB) , 14 RAEB in transformation (RAEB-t) , 3 already progressed to secondary AML.
  • RESULTS: The 3-year expected overall survival (OS) was 63.61%, 3-year expected disease-free survival ( DFS) 61.41%, and relapse rate 5.26%; OS for RA/ RAS, RAEB and RAEB-t/AML subgroup was 83.33%, 34.29% and 66.67% , respectively, and all had no statistic difference among them.


21. Zwierzina H, Suciu S, Loeffler-Ragg J, Neuwirtova R, Fenaux P, Beksac M, Harousseau J, Nuessler V, Cermak J, Solbu G, Willemze R, de Witte T, Amadori S, EORTC Leukemia Cooperative Group: Low-dose cytosine arabinoside (LD-AraC) vs LD-AraC plus granulocyte/macrophage colony stimulating factor vs LD-AraC plus Interleukin-3 for myelodysplastic syndrome patients with a high risk of developing acute leukemia: final results of a randomized phase III study (06903) of the EORTC Leukemia Cooperative Group. Leukemia; 2005 Nov;19(11):1929-33
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  • In this randomized phase III study of the EORTC Leukemia Cooperative Group, patients with myelodysplastic syndromes (MDS) with 10-30% bone marrow blasts and hematopoietic failure were treated with low-dose cytosine arabinoside (LD-AraC) (2 x 10 mg/m2/day subcutaneously (s.c.) days 1-14) either alone or in combination with rhGM-CSF or interleukin-3 (IL-3) both given s.c. at a dose of 150 microg/day from day 8 to 21.
  • A total of 180 evaluable patients with a median age of 65 years and refractory anemia with an excess of blasts (RAEB, n = 107) or RAEB in transformation (RAEBt, n = 73) were randomized.

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  • (PMID = 16151466.001).
  • [ISSN] 0887-6924
  • [Journal-full-title] Leukemia
  • [ISO-abbreviation] Leukemia
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / 5U10-CA11488-20; United States / NCI NIH HHS / CA / 5U10-CA11488-35
  • [Publication-type] Clinical Trial; Clinical Trial, Phase III; Journal Article; Multicenter Study; Randomized Controlled Trial; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Interleukin-3; 04079A1RDZ / Cytarabine; 83869-56-1 / Granulocyte-Macrophage Colony-Stimulating Factor
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22. Hasle H, Alonzo TA, Auvrignon A, Behar C, Chang M, Creutzig U, Fischer A, Forestier E, Fynn A, Haas OA, Harbott J, Harrison CJ, Heerema NA, van den Heuvel-Eibrink MM, Kaspers GJ, Locatelli F, Noellke P, Polychronopoulou S, Ravindranath Y, Razzouk B, Reinhardt D, Savva NN, Stark B, Suciu S, Tsukimoto I, Webb DK, Wojcik D, Woods WG, Zimmermann M, Niemeyer CM, Raimondi SC: Monosomy 7 and deletion 7q in children and adolescents with acute myeloid leukemia: an international retrospective study. Blood; 2007 Jun 1;109(11):4641-7
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  • We retrospectively collected data on 258 children with AML or refractory anemia with excess blasts in transformation (RAEB-T) and -7 or del(7q) with or without other cytogenetic aberrations +/- other].

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  • (PMID = 17299091.001).
  • [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
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23. Mihara K, Chowdhury M, Nakaju N, Hidani S, Ihara A, Hyodo H, Yasunaga S, Takihara Y, Kimura A: Bmi-1 is useful as a novel molecular marker for predicting progression of myelodysplastic syndrome and patient prognosis. Blood; 2006 Jan 1;107(1):305-8
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  • Higher positivity rate of Bmi-1 was preferentially seen in refractory anemia with excess blasts (RAEB), RAEB in transformation (RAEB-T), and MDS-AML compared with refractory anemia (RA) and RA with ringed sideroblasts (RARS).
  • Patients with RA and RARS with a higher percentage of Bmi-1+ cells showed disease progression to RAEB.
  • [MeSH-minor] Aged. Aged, 80 and over. Anemia, Refractory / diagnosis. Antigens, CD34. Biomarkers / analysis. Bone Marrow / chemistry. Bone Marrow / pathology. Case-Control Studies. Cell Proliferation. Disease Progression. Female. Humans. Leukemia, Myeloid. Male. Middle Aged. Polycomb Repressive Complex 1. Prognosis

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  • (PMID = 16160010.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD34; 0 / BMI1 protein, human; 0 / Biomarkers; 0 / Nuclear Proteins; 0 / Proto-Oncogene Proteins; 0 / Repressor Proteins; EC 6.3.2.19 / Polycomb Repressive Complex 1
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24. Breccia M, Mengarelli A, Mancini M, Biondo F, Gentilini F, Latagliata R, Mandelli F, Alimena G: Myelodysplastic syndromes in patients under 50 years old: a single institution experience. Leuk Res; 2005 Jul;29(7):749-54
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  • Patients demographics and clinical features at diagnosis were analysed for their prognostic value on survival and on risk of transformation to acute leukaemia.
  • According to FAB criteria there were 30 patients with refractory anaemia (RA), 3 with refractory anaemia with ringed sideroblasts (RARS), 18 with refractory anaemia with excess of blasts (RAEB), 6 with refractory anaemia with excess of blasts in transformation (RAEB-t) and 5 with chronic myelomonocytic leukaemia (CMML).
  • From univariate analysis we identified some features, which appeared to be predictive of outcome and risk of transformation to AML.
  • Age above 40 years (p = 0.002) and high risk according to IPSS score (p = 0.002) were found to be predictive for a shorter survival; FAB grouping (p = 0.0001), percentage > 5% of blasts in the bone marrow (p = 0.001) and high risk by IPSS score (p = 0.0003) were found to be predictive for a higher risk of transformation to AML.
  • [MeSH-minor] Adult. Anemia / etiology. Anemia, Sideroblastic / etiology. Cell Transformation, Neoplastic. Female. Humans. Leukemia / etiology. Male. Middle Aged. Prognosis. Retrospective Studies. Survival Analysis


25. Reddy KS: Double minutes (dmin) and homogeneously staining regions (hsr) in myeloid disorders: a new case suggesting that dmin form hsr in vivo. Cytogenet Genome Res; 2007;119(1-2):53-9
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  • We describe a case of an 87-year-old woman with myelodysplastic syndrome (MDS) in transformation.
  • At presentation, the patient suffered from refractory anemia with excess of blasts (RAEB).
  • After six months, the disease had progressed to refractory anemia with excess of blasts in transformation (RAEBT).

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  • [Copyright] Copyright (c) 2007 S. Karger AG, Basel.
  • (PMID = 18160782.001).
  • [ISSN] 1424-859X
  • [Journal-full-title] Cytogenetic and genome research
  • [ISO-abbreviation] Cytogenet. Genome Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
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26. Strom SS, Gu Y, Gruschkus SK, Pierce SA, Estey EH: Risk factors of myelodysplastic syndromes: a case-control study. Leukemia; 2005 Nov;19(11):1912-8
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  • The distribution by French-American-British (FAB) type was 67 (19%) refractory anemia (RA), 38 (11%) refractory anemia with ringed sideroblasts (RARS), 43 (12%) chronic myelomonocytic leukemia (CMML), 136 (38%) RA with excess blasts (RAEB), and 70 (20%) RAEB in transformation (RAEBT).
  • For RAEB/RAEBT cases, family history of hematopoietic cancer (OR = 2.10), smoking (OR = 1.52), and exposure to agricultural chemicals (OR = 3.79) or solvents (OR = 2.71) were independent risk factors.


27. Lessard M, Struski S, Leymarie V, Flandrin G, Lafage-Pochitaloff M, Mozziconacci MJ, Talmant P, Bastard C, Charrin C, Baranger L, Hélias C, Cornillet-Lefebvre P, Mugneret F, Cabrol C, Pagès MP, Fert-Ferret D, Nguyen-Khac F, Quilichini B, Barin C, Berger R, Groupe Francophone de Cytogénétique Hématologique (GFCH): Cytogenetic study of 75 erythroleukemias. Cancer Genet Cytogenet; 2005 Dec;163(2):113-22

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  • However, the large overlap of chromosomal abnormality patterns of EL (pure erythroid form excepted) and refractory anemia with excess of blasts in transformation (RAEB-t) favors the hypothesis of similarities between these 2 hematologic disorders.

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  • (PMID = 16337853.001).
  • [ISSN] 0165-4608
  • [Journal-full-title] Cancer genetics and cytogenetics
  • [ISO-abbreviation] Cancer Genet. Cytogenet.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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28. Varma N, Varma S: Proliferative indices, cytogenetics, immunophenotye and other prognostic parameters in myelodysplastic syndromes. Indian J Pathol Microbiol; 2008 Jan-Mar;51(1):97-101
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  • Thirty-five adult myelodysplastic syndrome (MDS) patients were included in this study: 11 refractory anemia (RA), 4 RA with ring sideroblasts (RARS), 9 RA with excess of blasts (RAEB), 10 RAEB in transformation (RAEB-T) and 1 chronic myelomonocytic leukemia (CMML).
  • Cytogenetic abnormalities were more frequent in RAEB (88.8%), RAEB-T (80.0%) and RA (63.6%) subtypes of MDS.
  • However, rest of the FAB subtypes were assigned into high, intermediate and low risk categories without any correlation with the survival and/or leukemic transformation.

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  • (PMID = 18417875.001).
  • [ISSN] 0377-4929
  • [Journal-full-title] Indian journal of pathology & microbiology
  • [ISO-abbreviation] Indian J Pathol Microbiol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] India
  • [Chemical-registry-number] 0 / Antigens, Nuclear; 0 / Biomarkers; 0 / Ki-67 Antigen; 0 / nucleolar organizer region associated proteins; 9007-73-2 / Ferritins; EC 1.1.1.27 / L-Lactate Dehydrogenase
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29. Nakai K, Kanda Y, Fukuhara S, Sakamaki H, Okamoto S, Kodera Y, Tanosaki R, Takahashi S, Matsushima T, Atsuta Y, Hamajima N, Kasai M, Kato S: Value of chemotherapy before allogeneic hematopoietic stem cell transplantation from an HLA-identical sibling donor for myelodysplastic syndrome. Leukemia; 2005 Mar;19(3):396-401
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • OS at 5 years was 57% for patients who underwent allo-SCT as a primary treatment for refractory anemia with excess blasts in transformation (RAEB-t) or secondary acute myeloid leukemia (AML) and 54% for those who underwent allo-SCT in remission after induction chemotherapy (P=0.81).
  • Although only a randomized controlled trial will be able to establish a definite conclusion, these results do not support the administration of induction chemotherapy for patients with RAEB-t or secondary AML before allo-SCT.


30. Mori T, Aisa Y, Yokoyama A, Nakazato T, Yamazaki R, Shimizu T, Mihara A, Kato J, Watanabe R, Takayama N, Ikeda Y, Okamoto S: Total body irradiation and granulocyte colony-stimulating factor-combined high-dose cytarabine as a conditioning regimen in allogeneic hematopoietic stem cell transplantation for advanced myelodysplastic syndrome: a single-institute experience. Bone Marrow Transplant; 2007 Feb;39(4):217-21
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • We evaluated 22 patients with advanced MDS, including refractory anemia with excess blasts (RAEB; n=10), RAEB in transformation (n=2), acute myelogenous leukemia transformed from MDS (n=6) and chronic myelomonocytic leukemia (n=4).


31. Chatterjee T, Mahapatra M, Dixit A, Naithani R, Tyagi S, Mishra P, Bhattacharya J, Dutta P, Pati HP, Choudhary DR, Kumar R, Choudhry VP, Saxena R: Primary myelodysplastic syndrome in children--clinical, hematological and histomorphological profile from a tertiary care centre in India. Hematology; 2005 Dec;10(6):495-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • A complete analysis of clinical features in conjunction with the bone marrow profile revealed 8 cases of refractory cytopenia (RC), 3 cases of refractory anemia with excess blasts (RAEB), 5 cases of refractory anemia with excess blasts in transformation (RAEB-T), 4 cases of Juvenile myelomonocytic leukemia (JMML) and a solitary cases of acute myeloid leukemia (AML) in Downs syndrome.
  • Three patients of RAEB-T progressed to AML within 3-4 months.
  • All 3 cases of RAEB are under regular follow-up and doing well.
  • Three cases of RAEB-T died (all had progressed to AML); the remaining 2 cases were lost to follow up.


32. Harada H, Harada Y, Kimura A: Implications of somatic mutations in the AML1/RUNX1 gene in myelodysplastic syndrome (MDS): future molecular therapeutic directions for MDS. Curr Cancer Drug Targets; 2006 Sep;6(6):553-65
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Recently, we reported the high incidence of somatic mutations in the AML1/RUNX1 gene, which is a critical regulator of definitive hematopoiesis and the most frequent target for translocation of AML, in MDS, especially refractory anemia with excess blasts (RAEB), RAEB in transformation (RAEBt) and AML following MDS (defined here as MDS/AML).


33. Raza A, Mehdi M, Mumtaz M, Ali F, Lascher S, Galili N: Combination of 5-azacytidine and thalidomide for the treatment of myelodysplastic syndromes and acute myeloid leukemia. Cancer; 2008 Oct 1;113(7):1596-604
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Six patients had refractory anemia (RA), 2 patients had RA with ringed sideroblasts, 10 patients had RA with excess blasts (RAEB), 1 patient had RAEB in transformation, 4 patients had chronic myelomonocytic leukemia, 1 patient had chronic idiopathic myelofibrosis, and 16 patients had AML.


34. Chen Y, Liu K, Xu L, Chen H, Liu D, Zhang X, Shi H, Han W, Wang Y, Zhao T, Wang J, Wang J, Huang X: HLA-mismatched hematopoietic SCT without in vitro T-cell depletion for myelodysplastic syndrome. Bone Marrow Transplant; 2010 Aug;45(8):1333-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • A total of 36 patients diagnosed with high-risk MDS (RAEB (refractory anemia with excess blasts) or RAEBt (RAEB in transformation)) underwent transplantation from HLA-mismatched family donors.

  • Genetic Alliance. consumer health - Myelodysplastic syndromes.
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  • (PMID = 20062100.001).
  • [ISSN] 1476-5365
  • [Journal-full-title] Bone marrow transplantation
  • [ISO-abbreviation] Bone Marrow Transplant.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 143011-72-7 / Granulocyte Colony-Stimulating Factor
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35. Girtovitis FI, Ntaios G, Papadopoulos A, Ioannidis G, Makris PE: Defective platelet aggregation in myelodysplastic syndromes. Acta Haematol; 2007;118(2):117-22
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • RAEB-t (refractory anemia with excess blasts in transformation) patients were most seriously affected.

  • Genetic Alliance. consumer health - Myelodysplastic syndromes.
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  • [Copyright] 2007 S. Karger AG, Basel
  • (PMID = 17726306.001).
  • [ISSN] 1421-9662
  • [Journal-full-title] Acta haematologica
  • [ISO-abbreviation] Acta Haematol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 1404-55-3 / Ristocetin; 61D2G4IYVH / Adenosine Diphosphate; 9007-34-5 / Collagen; YKH834O4BH / Epinephrine
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