Acquired cyclic thrombocytopenia-thrombocytosis with periodic defect of platelet function

Br J Haematol. 1993 Dec;85(4):718-22. doi: 10.1111/j.1365-2141.1993.tb03214.x.

Abstract

A periodic fall of platelet number characterizes an acquired pathological condition named cyclic thrombocytopenia. We observed a patient in whom the episodes of thrombocytopenia (platelet number less than 50 x 10(9)/l) were followed regularly by thrombocytosis (700-2300 x 10(9) platelets/l). The period of platelet count fluctuation was about 40 d. Morphological examination of bone marrow showed the cyclic disappearance of mature and immature megakaryocytes; bone marrow cultures revealed a periodic severe defect of both multilineage and single-lineage progenitor cell growth. When platelet count was falling, a mild defect of platelet aggregation and ATP release was observed, while platelet function was normal when platelet count was rising. Prednisone, thymopentin, high-dose intravenous gamma-globulin and splenectomy were without effect. After 4 years of cyclic platelet and megakaryocyte fluctuations, stable amegakaryocytic thrombocytopenia developed and the patient died of haemorrhagic stroke.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Blood Platelets / pathology
  • Blood Platelets / physiology*
  • Fatal Outcome
  • Humans
  • Male
  • Megakaryocytes / pathology
  • Periodicity
  • Platelet Aggregation
  • Platelet Count
  • Thrombocytopenia / physiopathology*
  • Thrombocytosis / physiopathology*