A critical review of the molecular pathophysiology of lenalidomide sensitivity in 5q-myelodysplastic syndromes

作者：Voutsadakis Ioannis A^*; Cairoli Anne

来源：Leukemia and Lymphoma, 2012, 53(5): 779-788.

DOI：10.3109/10428194.2011.623255

• Myelodysplastic syndromes
• lenalidomide
• 5q-syndrome
• haploinsufficiency
• NF-kappa B
• cell adhesion

The 5q deletion is a chromosomal abnormality that is observed in a subset of myelodysplastic syndromes (MDS). When isolated, this abnormality defines a specific clinical syndrome termed MDS associated with isolated deletion 5q, presenting with macrocytic anemia, normal platelet count or slight thrombocytosis, hypo-lobated megakaryocytes and fewer than 5% blasts in the bone marrow. MDS with the 5q deletion have a particular sensitivity to treatment with lenalidomide, a thalidomide analog. In this article, molecular changes in 5q- MDS derived from haploinsufficiency of genes encoded from the deleted region in 5q are reviewed, and mechanisms that link these molecular lesions with lenalidomide sensitivity are proposed.

• 出版日期2012-5