Discussion Author: George Spencer
Sickle cell disease (SCD) is a hereditary hemolytic anemia due to a defect in the beta hemoglobin chain that affects 1 in 600 African Americans. The deleterious sickling and resulting vaso-occlusive effects of sickle cell disease can affect nearly every organ system in the body. Its effect on the lungs, osseous structures, heart, upper abdomen (to include the gallbladder and spleen), can readily be appreciated on the chest radiograph. Sickle pulmonary disease in the form of acute chest syndrome (ACS) is the second most common complication and leading cause of death in SCD. ACS can manifest as a consolidation due to pneumonia, or as a diffuse airspace process due to infection or noncardiogenic pulmonary edema as a result of vaso-occlusion or fat embolism from distant infarcted bone. Chronic sickle pulmonary disease, i.e. pulmonary fibrosis – interstitial fibrosis on CT - and the subsequent development of pulmonary hypertension and cor pulmonale, is the result of repeated episodes of ACS. Cardiomegaly is the result of chronic hemolysis and its attendant high output failure. The incidence of pigmented gallstones is increased due to the effects of chronic hemolysis. An enlarged spleen may be the result of acute infarction. More commonly, the spleen shrinks and calcifies - easily recognized on CT - due to chronic, repetitive splenic microinfarctions, such that its shadow is barely visible on the chest radiograph. Sclerosis and flattening of humeral head and depressions in the end plates of vertebral bodies, i.e. the “H-shaped” deformity of vertebrae, are the result of bone infarcts (osteonecrosis).
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- Peumonia vs acute chest syndrome. Stigmata of sickle cell disease on the chest radiograph.
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