Clonal T cell-mediated cyclic thrombocytopenia

Br J Haematol. 2002 Dec;119(4):1059-61. doi: 10.1046/j.1365-2141.2002.03951.x.

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.

Publication types

  • Case Reports

MeSH terms

  • Cyclosporine / therapeutic use
  • Female
  • Gene Rearrangement, T-Lymphocyte
  • Humans
  • Immunophenotyping
  • Immunosuppressive Agents / therapeutic use
  • Leukocyte Count
  • Middle Aged
  • Periodicity*
  • Platelet Count
  • T-Lymphocyte Subsets / immunology*
  • Thrombocytopenia / blood
  • Thrombocytopenia / drug therapy
  • Thrombocytopenia / immunology*
  • Thrombopoietin / blood

Substances

  • Immunosuppressive Agents
  • Cyclosporine
  • Thrombopoietin