Clonal T cell-mediated cyclic thrombocytopenia |
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Authors: | Füreder Wolfgang Mitterbauer Gerlinde Thalhammer Renate Geissler Klaus Panzer Simon Krebs Michael Simonitsch-Klupp Ingrig Sperr Wolfgang R Lechner Klaus Kyrle Paul A |
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Affiliation: | Department of Internal Medicine I, Division of Haematology & Haemostaseology, The University of Vienna, Austria. vofuer@netway.at |
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Abstract: | Cyclic thrombocytopenia is a rare disorder characterized by periodic platelet count fluctuations of unknown aetiology. We report on a female patient with cyclic changes of platelet counts ranging from 6 x 10(9)/l to 753 x 10(9)/l in 4-week intervals. Platelet counts were inversely correlated to thrombopoietin levels suggesting production failure. Reticulocyte counts and neutrophil counts showed similar, but less prominent, fluctuations. Clonal T-cell receptor rearrangement was detected in bone marrow samples as well as in peripheral blood. Cell typing of blood lymphocytes revealed a relative increase in CD3+ T cells. Treatment with cyclosporine A resulted in a substantial improvement of platelet counts. Taken together, we provide evidence for clonal T-cell mediated bone marrow failure with cyclic impairment of thrombopoiesis responsive to cyclosporine therapy. |
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Keywords: | cyclic thrombocytopenia T-cell receptor thrombopoietin cyclosporine |
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