Document Detail


Clinical management of myelodysplastic syndromes with interstitial deletion of chromosome 5q.
MedLine Citation:
PMID:  16735711     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Deletions of the long (q) arm of chromosome 5 [del(5q)]occur in patients with myelodysplastic syndromes (MDS) including, but not limited to, those who meet the WHO definition of the 5q- syndrome. Del(5q) MDS patients frequently have symptomatic anemia, and its treatment has traditionally consisted of RBC transfusions and, for some, iron chelation therapy. Erythropoietin, darbepoetin, hypomethylating agents, and lenalidomide can enhance erythropoiesis in MDS patients with anemia, increasing hemoglobin levels and abrogating RBC transfusion requirements. Lenalidomide is particularly active in treating the anemia of del(5q) MDS, which is especially relevant given the low response rate to erythropoietin in this group of patients. In a recent study of 43 MDS patients, 10 of 12 patients (83%) with del(5q) MDS achieved sustained RBC transfusion independence (or a > 2 g/dL increase in hemoglobin), compared with 57% of those with a normal karyotype and 12% of those with other karyotypic abnormalities. Complete cytogenetic remissions were achieved in 75% (nine of 12) of the del(5q) MDS patients, suggesting that lenalidomide targets a fundamental pathogenetic feature of MDS that is more pronounced in the presence of chromosomal 5q deletions. This review highlights some issues about the classification and treatment of del(5q) MDS.
Authors:
Stephen D Nimer
Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Journal of clinical oncology : official journal of the American Society of Clinical Oncology     Volume:  24     ISSN:  1527-7755     ISO Abbreviation:  J. Clin. Oncol.     Publication Date:  2006 Jun 
Date Detail:
Created Date:  2006-05-31     Completed Date:  2006-06-12     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  8309333     Medline TA:  J Clin Oncol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  2576-82     Citation Subset:  IM    
Affiliation:
Division of Hematologic Oncology, Memorial Sloan-Kettering Cancer Center, New York, NY 10021-6007, USA. s-nimer@mskcc.org
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MeSH Terms
Descriptor/Qualifier:
Anemia, Hypochromic / drug therapy,  etiology,  pathology,  therapy*
Azacitidine / therapeutic use
Bone Marrow / pathology
Chromosome Aberrations
Chromosomes, Human, Pair 5*
Erythrocyte Transfusion
Erythropoiesis / drug effects
Erythropoietin / therapeutic use
Gene Deletion*
Hematinics / therapeutic use*
Hematopoietic Stem Cell Transplantation
Humans
Karyotyping
Myelodysplastic Syndromes / classification,  complications,  drug therapy,  genetics*,  pathology,  therapy*
Predictive Value of Tests
Prognosis
Randomized Controlled Trials as Topic
Recombinant Proteins / therapeutic use
Thalidomide / analogs & derivatives*,  therapeutic use
Chemical
Reg. No./Substance:
0/Hematinics; 0/Recombinant Proteins; 11096-26-7/Erythropoietin; 191732-72-6/lenalidomide; 320-67-2/Azacitidine; 50-35-1/Thalidomide

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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