Allogeneic bone marrow transplantation for severe post-splenectomy thrombophilic state in leaky red cell membrane haemolytic anaemia of the stomatocytosis class.
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.
Bergheim
Jann
J
Section of Haematology, Department of Medicine, Haukeland Hospital, Bergen, Norway.
Ernst
Peter
P
Brinch
Lorentz
L
Gore
D M
DM
Chetty
M C
MC
Stewart
G W
GW
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
D012964:Sodium; D011188:Potassium