Coronary thrombosis in a patient with May-Hegglin anomaly.
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.
McDunn
S
S
Department of Medicine, Northwestern University Medical School, Chicago, Illinois.
Hartz
W
W
Ts'Ao
C
C
Green
D
D
D001792:Blood Platelets / Q000473:pathology* / Q000502:physiology; D001810:Blood Volume / Q000502:physiology; D003328:Coronary Thrombosis / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D005260:Female; D006402:Hematologic Diseases / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D002479:Inclusion Bodies / Q000648:ultrastructure*; D008854:Microscopy, Electron; D008875:Middle Aged; D009504:Neutrophils / Q000473:pathology* / Q000648:ultrastructure; D010974:Platelet Aggregation / Q000502:physiology; D010976:Platelet Count