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Durable Hematological and Major Cytogenetic Response in a Patient with Isolated 20q Deletion Myelodysplastic Syndrome Treated with Lenalidomide

DOI: 10.1155/2014/949515

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Abstract:

Myelodysplastic syndrome (MDS) is a clonal bone marrow disorder characterized by ineffective hematopoiesis. It is characterized by peripheral blood cytopenia and significant risk of progression to acute myeloid leukemia result. Deletion of the long arm of chromosome 20 (20q deletion) is present in 3–7% of patients with MDS. Lenalidomide is an immunomodulatory agent with antiangiogenic activity. It is FDA approved for the treatment of anemia in patients with low or int-1 risk MDS with chromosome 5q deletion with or without additional cytogenetic abnormalities. Study of lenalidomide in patients with MDS without 5q deletion but other karyotypic abnormalities demonstrated meaningful activity in transfusion dependent patients; however, response of patients with isolated 20q deletion to lenalidomide is not known. We are reporting a patient with 20q deletion MDS treated with lenalidomide after he failed to respond to azacytidine; to our knowledge this is the first report of a patient with isolated 20q deletion treated with lenalidomide. 1. Introduction Myelodysplastic syndrome (MDS) is a clonal bone marrow disorder characterized by ineffective hematopoiesis. The FDA approved lenalidomide for the treatment of anemia in patients with low or int-1 risk MDS with chromosome 5q deletion with or without additional cytogenetic abnormalities. Response of patients with isolated 20q deletion to lenalidomide is not known. We are reporting a patient with 20q deletion MDS treated with lenalidomide after he failed to respond to azacytidine; to our knowledge this is the first report of a patient with isolated 20q deletion treated with lenalidomide. 2. Case Report A 67-year-old man presented with feeling progressively weaker for few weeks. He noticed easy bruising several days prior to presentation. No overt bleeding or fever was reported. Patient was unable to function due to progressive weakness. Complete blood count revealed pancytopenia with WBC count of 1.6?M/mcL, hemoglobin of 8.6?g/dL, and platelet count of 53?K/mcL. absolute neutrophil count (ANC) was 600?K/mcL. MCV was 99.0?fL. Bone marrow aspirate and biopsy revealed increased myeloid blasts suggestive of high-grade myelodysplastic syndrome (as shown in Figures 1(a) and 1(b)). Flow cytometric analysis of bone marrow showed increased myeloid blasts expressing dim CD45, CD13, dim CD33, CD34, CD117, and HLA-DR (as shown in Figures 2(a) and 2(b)). Blasts represented 11% of marrow cellular events. IPSS cumulative score of 2 was determined by 11–20% of blasts (1.5) and 2-3 cytopenias (0.5). MDS and MLL FISH revealed

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