Autosplenectomy is feature of

Ans. b (Sickle cell disease) (Ref. H - 18th/Ch. 104)Surgical removal of the spleen is an obvious cause of hyposplenism. Patients with sickle cell disease often suffer from autosplenectomy as a result of splenic destruction by the numerous infarcts associated with sickle cell crises during childhood. Indeed, the presence of a palpable spleen in a patient with sickle cell disease after age 5 suggests a coexisting hemoglobinopathy, e.g., thalassemia or hemoglobin C. In addition, patients who receive splenic irradiation for a neoplastic or autoimmune disease are also functionally hyposplenic. The term hyposplenism is preferred to asplenism in referring to the physiologic consequences of splenectomy because asplenia is a rare, specific, and fatal congenital abnormality in which there is a failure of the left side of the coelomic cavity (which includes the splenic anlagen) to develop normally. Infants with asplenia have no spleens, but that is the least of their problems. The right side of the developing embryo is duplicated on the left so there is liver where the spleen should be, there are two right lungs, and the heart comprises two right atria and two right ventricles.