HEREDITARY SPHEROCYTOSIS, THROMBOCYTOSIS, AND CHRONIC PULMONARY EMBOLI - A CASE-REPORT AND REVIEW OF THE LITERATURE

Thrombocytosis in post-splenectomy patients with hereditary spherocyto sis (HS) is usually not attended by an increased risk of thrombosis. R eview of the literature revealed HS in association with pulmonary thro mbosis, portal vein thrombosis, and cerebral infarction in two brother s, TTP in an asplenic patient and a patient with corpora cavernosum th rombosis causing segmental priapism. We report a case of a 30-year-old white male with HS who presented with hemoptysis 29 years after splen ectomy. Work-up revealed a hypercoagulable state with thrombocytosis a nd recurrent pulmonary emboli resulting in severe pulmonary hypertensi on, cor pulmonale, atrial flutter, and syncope. (C) 1998 Wiley-Liss, I nc.