Platelet Studies in the Pathogenesis of Thrombocytopenia in May-Hegglin Anomaly

Thrombocytopenia has a variable incidence in the May-Hegglin anomaly, a rare congenital syndrome characterized by inclusions resembling Dohle bodies within leukocytes, thrombocytopenia, and giant platelets. We studied a 16-year-old girl and three relatives with the syndrome to elucidate the pathogenesis of the thrombocytopenia and the degree of impaired hemostasis. On confirmation of automated platelet counts by manual techniques we found that...