Increased destruction of red blood cells by the reticuloendothelial system, in particular by the spleen, results in its enlargement (splenomegaly). Many patients with a hemoglobinopathy have undergone splenectomy. The main therapeutic rationale for splenectomy in transfusion-dependent patients has been to decrease red blood cell consumption and transfusion requirements with the ultimate goal of reducing iron overload. However, the current transfusion regimens are setting more accurate pre-transfusion hemoglobin levels and more correct intervals between transfusions, which has considerably reduced the incidence of splenomegaly and splenectomy.

Throughout the care of the patient with hemoglobinopathy, the size of the spleen should be carefully monitored on physical examination and, as needed, by ultrasonography.

All guidelines agree that physicians should adopt a guarded approach and restrict splenectomy to certain indications, in view of the observation of an increased risk of venous thrombosis and pulmonary hypertension, alongside with overwhelming infections after splenectomy.