Ten families, 39 cases of the May-Hegglin anomaly have been reported to date. The present report deals with the case of this anomaly, presumably the first description in Japan.
The propositus was a 26-year-old male who had in good health except an episode of excess post operative bleeding at the time of maxillary sinectomy, until he consulted us for upper abdominal discomfort and melena. Diagnosis of malignant tumor of the stomach was made by the X-ray and the endoscopic examination. Surgical operation was successfully performed and the histological diagnosis of the removed tumor was “carcinoma simplex”.
Examination of peripheral blood smear revealed the characteristic features of the May-Hegglin anomaly, i.e., the presence of the leukocytic inclusion bodies and the giant platelet.
Leukocytic inclusion bodies (Döhle body), usually crescent, fusiform or spindle-like in shape, were persisted after the removal of the malignant tumor. Basophilic and pyroninophilic properties of the inclusion body were lost after RNAase treatment. They appeared in neutrophils, eosinophils and basophils, but not in lymphocytes. They also appeared in monocytes, although they were less frequent and distinct.
Moderate thrombocytopenia and the presence of cigarshaped giant platelet was detected at the admission and throughout the observation period. Succeeding examinatian revealed mild prolongation of bleeding time and coagulation time, but definite defect of coagulation factors were not confirmed.
Family study was performed on 20 blood relatives. Typical leukocytic inclusion bodies and giant platelet were detected in father and sister of the propositus. All of the remaining members were negative.
Chromosomal analysis of the propositus, both direct and indirect methods, failed to confirm any morphological abnormality.
