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Case Rep Hematol. 2013;2013:718480. doi: 10.1155/2013/718480. Epub 2013 Apr 7.

Refractory anemia with ring sideroblasts associated with marked thrombocytosis complicated by massive splenomegaly treated with lenalidomide resulting in resolution of splenomegaly but severe and prolonged pancytopenia.

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1
Department of Haematology, Aberdeen Royal Infirmary, Aberdeen AB25 2ZN, UK.

Abstract

Refractory anemia with ring sideroblasts associated with marked thrombocytosis (RARS-T) is a hematological malignancy that combines features of both a myeloproliferative and myelodysplastic disorder. There have been recent reports of the successful treatment of anemia in 2 patients with RARS-T with lenalidomide. Here we report the successful treatment of massive splenomegaly in a patient with a long history of RARS-T resulting in complete resolution of splenomegaly, but with prolonged severe cytopenias. We also report the acquisition of the t(3;12)(q26;p13) translocation previously described in cases of myelodysplasia and the potential for transformation to myelofibrosis.

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