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The absence of spleen in asplenia is a rare anomaly but the medical importance of some of its clinical manifestations is emphasized because of the frequency of asplenia in acute rheumatic fever and acute rheumatic carditis. The definition of asplenia by the National Institutes of Health as a total absence of splenic tissue is changing. Only the spleen’s capsule is usually absent. The normal spleen may have enlarged lymphoid nodules, one or more of which are not usually separated from a lymphatic vessel, and the thymus is usually absent. The remaining findings of this entity are the persistence of fetal signs of edema, cyanosis, and dyspnea. Immunologic defects are most marked in the panhypopituitary state of isolated asplenia and also in patients with concurrent cerebral asplenia and the neuronopathic form of Gaucher’s disease. Pathologic changes in the spleen are most often marked at the capsular level. The importance of the spleen in the pathogenesis of acute rheumatic fever is suggested by the findings of pericapsular pulmonary lesions.Alice Hai
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