Large granular lymphocyte leukemia presenting with both amegakaryocytic thrombocytopenic purpura and pure red cell aplasia: Clinical course and response to immunosuppressive therapy

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Abstract
Large granular lymphocyte (LGL) leukemia is typically associated with neutropenia and occasionally pure red cell aplasia. We now report a new association of LGL leukemia with amegakaryocytic aplasia that was also accompanied by pure red cell aplasia. An 82-year-old female presented with a platelet count of 16,000/μl at the time of a gastrointestinal bleed. The white blood cell count was within normal limits but there was a relative lymphocytosis of large granulated cells that by flow cytometric and gene rearrangement studies was consistent with a monoclonal process. Platelet and red cell transfusion dependence persisted over 17 months with numerous immunosuppressive treatments given including prednisone, cyclophosphamide, vincristine, cyclosporine, gammaglobulin, and azathioprine. Presently, 36 months since the time of presentation, she is in a clinical remission with follow-up flow cytometric and gene rearrangement studies of the peripheral blood without definite evidence of the LGL clone. This case report illustrates the various therapeutic approaches to be considered in LGL leukemia and is a reminder that severe and life-threatening thrombocytopenia can be a feature of LGL leukemia unlike the usual course of LGL leukemia with neutropenia as the major clinical feature.