Successful splenectomy in May-Hegglin anomaly: report of a case with platelet kinetic studies

Bleeding diathesis is not necessarily one of the conspicuous and serious characteristics of the macrothrombocytopenic syndromes, which include the May-Hegglin anomaly. Nevertheless, occasionally prolonged bleeding time may endanger organs or even life. We report a case of a young girl with severe thrombocytopenia due to May-Hegglin anomaly, who was facing blindness because of intraocular haemorrhages. Being unable to intervene in the pathogenesis of the disease, we performed splenectomy after proving splenic sequestration of platelets. This procedure allowed the performance of a successful bilateral vitrectomy.