The role of splenectomy in the management of hypersplenism arising in beta-thalassaemias is well established. Its indications in Cooley's anaemia are now more clearly determined with the Transfusion Quotient (T.Q.). This does not apply to the thalassaemia intermedia syndromes because of the small number of these patients followed in each center and the genetic variety of this group. Among 21 splenectomized beta-thalassaemic patients (including 4 with beta-thalassaemia intermedia), 18 underwent surgery for hypersplenism, 2 for a severe immune hemolysis, and one, as an emergency, for splenic infarction. All patients improved by the treatment. The best results, but also the only serious early complications, were encountered in the thalassaemia intermedia group. Septic complications were absent in our series except for one case of meningitis in a Cooley patient, 6 months after surgery. Finally, among the pool of beta-thalassaemic patients from which our group was extracted, no patients treated with a high blood transfusion regimen, developed hypersplenism and required spelenectomy.
|Number of pages||2|
|Journal||IRCS Medical Science|
|Publication status||Published - 1983|
ASJC Scopus subject areas
- Biochemistry, Genetics and Molecular Biology(all)