Coronary thrombosis in a patient with May-Hegglin anomaly

Am J Clin Pathol. 1991 May;95(5):715-8. doi: 10.1093/ajcp/95.5.715.

Abstract

May-Hegglin anomaly (MHA) is a rare hereditary condition that is characterized by cytoplasmic inclusions in leukocytes and giant platelets. Many patients have some degree of thrombocytopenia. Most individuals with MHA are asymptomatic, but 25-43% of patients previously reported have had a hemorrhagic tendency. The authors describe a patient with MHA who had no history of hemorrhage but who developed complete coronary thrombosis after attempted angioplasty despite an apparent platelet count of 24,000 per mm3. Laboratory investigations revealed a normal bleeding time, normal platelet aggregation, and an increase in the size of approximately two-thirds of the platelets. The calculated platelet mass was near normal, which probably explains the thrombosis despite a decrease in platelet numbers. The authors conclude that in some patients with MHA platelets are functionally active both in vivo and in vitro.

Publication types

  • Case Reports

MeSH terms

  • Blood Platelets / pathology*
  • Blood Platelets / physiology
  • Blood Volume / physiology
  • Coronary Thrombosis / etiology*
  • Coronary Thrombosis / pathology
  • Coronary Thrombosis / physiopathology
  • Female
  • Hematologic Diseases / complications*
  • Hematologic Diseases / pathology
  • Hematologic Diseases / physiopathology
  • Humans
  • Inclusion Bodies / ultrastructure*
  • Microscopy, Electron
  • Middle Aged
  • Neutrophils / pathology*
  • Neutrophils / ultrastructure
  • Platelet Aggregation / physiology
  • Platelet Count