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THROMBO‐EMBOLIC DISEASE AFTER SPLENECTOMY FOR HEREDITARY STOMATOCYTOSIS
Author(s) -
Stewart Gordon W.,
Amess John A. L.,
Eber Stefan W.,
Kingswood Christopher,
Lane Peter A.,
Smith Brian D.,
Mentzer William C.
Publication year - 1996
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.1365-2141.1996.4881033.x
Subject(s) - splenectomy , medicine , disease , spleen
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 be 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.