[Splenectomy in hereditary hemolytic anemia: 82 Tunisian cases]

ABSTRACT

Splenectomy is frequently advised in hereditary hemolytic anemia.Severe complications could occur after splenectomy. To provide the indication and benefit of splenectomy clinical and biological patterns were performed in a retrospective study of 82 patients 17 homozygous beta thalassemia, 17 thalassemia intermedia, 33 heterozygote HbS I beta thalassemia and 15 hereditary spherocytosis. Splenectomy was performed for Hypertransfusion in homozygous thalassemia, hereditary spherocytosis; hypersplenism in Thalassemia intermedia and splenic sequestration in heterozygote HbS/beta thalassemia.The benefit of splenectomy was proved in hereditary spherocytosis [100%], heterozygote HbS/beta thalassemia [90%] and thalassemia intermedia [75%];nevertheless in homozygous beta thalassemia.Post splenectomical complication are often thrombocytosis, thrombosis and infections. Splenectomy should be performed in hereditary hemolytic anemia to reduce and avoid transfusion