Allogeneic bone marrow transplantation for severe post-splenectomy thrombophilic state in leaky red cell membrane haemolytic anaemia of the stomatocytosis class

Br J Haematol. 2003 Apr;121(1):119-22. doi: 10.1046/j.1365-2141.2003.04262.x.

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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Anemia, Hemolytic / metabolism
  • Anemia, Hemolytic / surgery
  • Anemia, Hemolytic / therapy*
  • Bone Marrow Transplantation*
  • Erythrocyte Membrane / metabolism
  • Fatal Outcome
  • Female
  • Humans
  • Iron Overload
  • Potassium / metabolism
  • Pulmonary Embolism / etiology
  • Pulmonary Embolism / metabolism
  • Sodium / metabolism
  • Splenectomy
  • Thrombophilia / metabolism
  • Thrombophilia / therapy*
  • Transplantation, Homologous

Substances

  • Sodium
  • Potassium