A Case of May-Hegglin Anomaly

Abstract

A seventeen-year-old male was examined for frequent occurrences of epistaxis which he had had since the age of seven. The examination revealed marked decrease of thrombocytes in the peripheral blood and slight increase of megakaryocytes in the bone marrow. On the presumptive diagnosis of idiopathic thrombocytopenic purpura he was started on prednisolone and 6-mercaptopurine. Since the medication did not improve his conditon, the splenectomy was perfomed which neither raised his platelet count, nor cured frequent attacks of the epistaxis. Subsequent detailed examinations of his peripheral blood smears disclosed giant platelets and Döhle bodies in the polymorphonuclear leukocytes. The giant platelets were observed also in the farther and brother of the patient.
Thus, the ultimate diagnosis of this patient was considered to be May-Hegglin anomaly. In this report we have also described the electron-microscopic observations on Döhle bodies and giant platelets seen in this case.