Large granular lymphocytic proliferation-associated cyclic thrombocytopenia

Am J Hematol. 2005 Aug;79(4):334-6. doi: 10.1002/ajh.20375.

Abstract

Cyclic thrombocytopenia is a rare condition characterized by regular fluctuations in the platelet count, resulting in bleeding at the time of platelet count nadir. We evaluated a male patient whose platelet count cycled between <10x10(9)/L and a maximum of >1300x10(9)/L over a median of every 42 days (range, 28-57 days). Antiplatelet antibodies were present at highest titer just prior to platelet nadirs. A pathologic expansion of CD3+CD57+ large granular lymphocytes (LGLs) along with a clonal rearrangement of the T-cell receptor (TCR) gamma gene in circulating T cells was detected. LGLs decreased in response to treatment with cyclosporine-A (CsA), but the cycling of the platelet count continued. This is the first report of cyclic thrombocytopenia associated with a T-LGL lymphoproliferative disorder.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor
  • Humans
  • Lymphoproliferative Disorders / blood
  • Lymphoproliferative Disorders / complications
  • Lymphoproliferative Disorders / genetics
  • Lymphoproliferative Disorders / immunology*
  • Male
  • Platelet Count
  • Purpura, Thrombocytopenic / blood
  • Purpura, Thrombocytopenic / complications
  • Purpura, Thrombocytopenic / immunology*
  • Purpura, Thrombocytopenic / therapy
  • T-Lymphocyte Subsets / immunology*