title: Allogeneic bone marrow transplantation for severe post-splenectomy thrombophilic state in leaky red cell membrane haemolytic anaemia of the stomatocytosis class.
abstract: The tendency for thrombosis to occur if haemolysis persists after splenectomy is especially marked in "hereditary stomatocytosis", in which the red cell membrane "leaks" Na and K. A 21-year-old woman, who was splenectomized in childhood for a congenital haemolytic state, presented with major pulmonary embolism that recurred despite anticoagulation. Tests showed a significant cation leak with a "shallow-slope" abnormality in temperature dependence. Allogeneic bone marrow transplantation caused the thrombophilic state to cease and subsequently anticoagulation was stopped without recurrence of thromboembolism. However, she died 9 months after transplantation: iron overload, intensified by the transfusion demands of the transplant, was a major factor.
authors.affiliation: Section of Haematology, Department of Medicine, Haukeland Hospital, Bergen, Norway.
authors.forename: Jann
authors.initials: J
authors.lastname: Bergheim
authors.forename: Peter
authors.initials: P
authors.lastname: Ernst
authors.forename: Lorentz
authors.initials: L
authors.lastname: Brinch
authors.forename: D M
authors.initials: DM
authors.lastname: Gore
authors.forename: M C
authors.initials: MC
authors.lastname: Chetty
authors.forename: G W
authors.initials: GW
authors.lastname: Stewart
mesh_terms: D000328:Adult; D000743:Anemia, Hemolytic / Q000378:metabolism / Q000601:surgery / Q000628:therapy*; D016026:Bone Marrow Transplantation; D004910:Erythrocyte Membrane / Q000378:metabolism; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D019190:Iron Overload; D011188:Potassium / Q000378:metabolism; D011655:Pulmonary Embolism / Q000209:etiology / Q000378:metabolism; D012964:Sodium / Q000378:metabolism; D013156:Splenectomy; D019851:Thrombophilia / Q000378:metabolism / Q000628:therapy*; D014184:Transplantation, Homologous
chemical_list: D012964:Sodium; D011188:Potassium