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Allogeneic bone marrow transplantation for severe post‐splenectomy thrombophilic state in leaky red cell membrane haemolytic anaemia of the stomatocytosis class
Author(s) -
Bergheim Jann,
Ernst Peter,
Brinch Lorentz,
Gore D. M.,
Chetty M. C.,
Stewart G. W.
Publication year - 2003
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1046/j.1365-2141.2003.04262.x
Subject(s) - medicine , splenectomy , haemolysis , surgery , transplantation , thrombosis , immunology , spleen
Summary. 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.