What is the role of splenectomy in the treatment of beta thalassemia?

Updated: Dec 19, 2018
  • Author: Pooja Advani, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Physicians often use splenectomy to decrease transfusion requirements in patients with thalassemia major. (Patients with thalassemia minor only rarely require splenectomy.) Splenectomy is recommended when the calculated annual transfusion requirement is greater than 200-220 mL RBCs/kg/y with a hematocrit value of 70%. [12] In addition to reducing transfusion requirements and the resultant iron overload, splenectomy also prevents extramedullary hematopoiesis.

Because postsplenectomy sepsis is possible, defer this procedure until the patient is older than 6-7 years. In addition, to minimize the risk of postsplenectomy sepsis, vaccinate the patient against Pneumococcus species, Meningococcus species, and Haemophilus influenzae. Administer penicillin prophylaxis to children after splenectomy. Postsplenectomy thrombocytosis can increase the risk of thrombotic events. The risk-to-benefit ratio for this procedure should be cautiously evaluated.


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