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 pulmona...

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Bibliographic Details
Published in:British journal of haematology 2003-04, Vol.121 (1), p.119-122
Main Authors: Bergheim, Jann, Ernst, Peter, Brinch, Lorentz, Gore, D. M., Chetty, M. C., Stewart, G. W.
Format: Article
Language:English
Subjects:
Adult
Anemia, Hemolytic - metabolism
Anemia, Hemolytic - surgery
Anemia, Hemolytic - therapy
Anemias. Hemoglobinopathies
Biological and medical sciences
Bone Marrow Transplantation
cation leak
Diseases of red blood cells
Erythrocyte Membrane - metabolism
Fatal Outcome
Female
Hematologic and hematopoietic diseases
Hematology
Humans
Iron Overload
Medical sciences
Potassium - metabolism
Pulmonary Embolism - etiology
Pulmonary Embolism - metabolism
Sodium - metabolism
Splenectomy
stomatocytosis
Thrombophilia - metabolism
Thrombophilia - therapy
thrombosis
Transplantation, Homologous
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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.
ISSN:0007-1048
1365-2141
DOI:10.1046/j.1365-2141.2003.04262.x