title: Thrombo-embolic disease after splenectomy for hereditary stomatocytosis.
abstract: 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.
authors.affiliation: Department of Medicine, University College London Medical School, UK.
authors.forename: G W
authors.initials: GW
authors.lastname: Stewart
authors.forename: J A
authors.initials: JA
authors.lastname: Amess
authors.forename: S W
authors.initials: SW
authors.lastname: Eber
authors.forename: C
authors.initials: C
authors.lastname: Kingswood
authors.forename: P A
authors.initials: PA
authors.lastname: Lane
authors.forename: B D
authors.initials: BD
authors.lastname: Smith
authors.forename: W C
authors.initials: WC
authors.lastname: Mentzer
mesh_terms: D000328:Adult; D000368:Aged; D000745:Anemia, Hemolytic, Congenital / Q000235:genetics / Q000601:surgery*; D005260:Female; D006486:Hemosiderosis / Q000209:etiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D011655:Pulmonary Embolism / Q000209:etiology; D013156:Splenectomy / Q000009:adverse effects*; D013923:Thromboembolism / Q000209:etiology*; D013924:Thrombophlebitis / Q000209:etiology*