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

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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.

Original languageEnglish
Pages (from-to)2576-2582
Number of pages7
JournalJournal of Clinical Oncology
Volume24
Issue number16
DOIs
StatePublished - Jun 1 2006
Externally publishedYes

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Chromosome Deletion
Myelodysplastic Syndromes
Anemia
Erythropoietin
Hemoglobins
Chelation Therapy
Chromosomes, Human, Pair 5
Erythropoiesis
Karyotype
Cytogenetics
Chromosome 5q Deletion Syndrome
Iron

ASJC Scopus subject areas

  • Cancer Research
  • Oncology

Cite this

Clinical management of myelodysplastic syndromes with interstitial deletion of chromosome 5q. / Nimer, Stephen D.

In: Journal of Clinical Oncology, Vol. 24, No. 16, 01.06.2006, p. 2576-2582.

Research output: Contribution to journalArticle

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