Clinical management of myelodysplastic syndromes with interstitial deletion of chromosome 5q Journal Article


Author: Nimer, S. D.
Article Title: Clinical management of myelodysplastic syndromes with interstitial deletion of chromosome 5q
Abstract: 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. © 2006 by American Society of Clinical Oncology.
Keywords: treatment response; gene deletion; genetics; antianemic agent; lenalidomide; thalidomide; clinical feature; clinical trial; pathogenesis; review; comparative study; controlled clinical trial; classification; erythropoietin; bone marrow; erythropoiesis; randomized controlled trial; cytogenetics; stem cell transplantation; hematopoietic stem cell transplantation; drug effect; pathology; chromosome aberration; myelodysplastic syndrome; randomized controlled trials; recombinant proteins; recombinant protein; prediction and forecasting; predictive value of tests; drug derivative; chromosome aberrations; chromosome deletion; erythrocyte transfusion; karyotyping; azacitidine; myelodysplastic syndromes; iron chelation; novel erythropoiesis stimulating protein; chromosome 5; chromosome 5q; chromosomes, human, pair 5; hematinics; iron deficiency anemia; anemia, hypochromic
Journal Title: Journal of Clinical Oncology
Volume: 24
Issue: 16
ISSN: 0732-183X
Publisher: American Society of Clinical Oncology  
Date Published: 2006-06-01
Start Page: 2576
End Page: 2582
Language: English
DOI: 10.1200/jco.2005.03.6715
PUBMED: 16735711
PROVIDER: scopus
DOI/URL:
Notes: --- - "Cited By (since 1996): 31" - "Export Date: 4 June 2012" - "CODEN: JCOND" - "Source: Scopus"
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  1. Stephen D Nimer
    347 Nimer