British Journal of Haematology · 1996 · 208 citations · 0 references
MedicineHematologySurgeryHematopathologyHemostasisIron Metabolism DisordersAnemiaHematologic DisordersHemostatic DisordersThrombosisThrombotic DisordersVenous ThromboembolismHereditary Bleeding DisordersPulmonary Venous HypertensionSplenic Iron OverloadSurgery-associated Venous ThromboembolismTherapeutic Risk-benefit Assessment
Nine cases of hereditary stomatocytosis (HSt) are presented which show documented thrombotic complications after splenectomy. In three cases, patients became severely ill with pulmonary hypertension and a fourth developed portal hypertension. One unsplenectomized affected adult relative had suspected but unconfirmed thrombotic pathology; the six other affected unsplenectomized adults did not. Since splenectomy is of only limited therapeutic benefit in stomatocytosis, it should not be performed without careful consideration. A tendency to iron overload, even without hypertransfusion and irrespective of splenectomy, is evident in many of these patients.