[Splenectomy in hereditary hemolytic anemia: 82 Tunisian cases].

Background: Splenectomy is frequently advised in hereditary hemolytic anemia. Severe complications could occur after splenectomy.

Aim: To provide the indication and benefit of splenectomy

Methods: clinical and biological patterns were performed in a retrospective study of 82 patients: 17 homozygous beta thalassemia, 17 thalassemia intermedia, 33 heterozygote Hb/S beta thalassemia and 15 hereditary spherocytosis.

Results: 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.

Conclusion: Splenectomy should be performed in hereditary hemolytic anemia to reduce and avoid transfusion.