Thrombo-embolic disease after splenectomy for hereditary stomatocytosis

Br J Haematol. 1996 May;93(2):303-10. doi: 10.1046/j.1365-2141.1996.4881033.x.


Nine cases of hereditary stomatocytosis (HSt) are presented which show documented thrombotic complications after splenectomy. In three cases, patients became severely ill with pulmonary hypertension and a fourth developed portal hypertension. One unsplenectomized affected adult relative had suspected but unconfirmed thrombotic pathology; the six other affected unsplenectomized adults did not. Since splenectomy is of only limited therapeutic benefit in stomatocytosis, it should not be performed without careful consideration. A tendency to iron overload, even without hypertransfusion and irrespective of splenectomy, is evident in many of these patients.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Anemia, Hemolytic, Congenital / genetics
  • Anemia, Hemolytic, Congenital / surgery*
  • Female
  • Hemosiderosis / etiology
  • Humans
  • Male
  • Middle Aged
  • Pedigree
  • Pulmonary Embolism / etiology
  • Splenectomy / adverse effects*
  • Thromboembolism / etiology*
  • Thrombophlebitis / etiology*