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Everything you need to know about Computed Tomography (CT) & CT Scanning

MDCT of the Spleen: Sickle Cell Disease


 Sickle cell disease is a hemoglobinopathy caused by a genetic mutation producing an abnormal Beta chain in hemoglobin S. This mutation causes deformation of erythrocytes when in the deoxygenated state. The erythrocytes distort into a sickle configuration that hinders their passage through the capillary network, causing ischemia and infarction in the organs involved.

Clinically, patients in crisis have severe pain related to the organ being affected. Most commonly the disease affects the spleen, lungs, kidneys, brain and bone. Individuals often develop gallstones, and multiorgan infarctions. These patients can therefore suffer from cardiomyopathy, osteomyelitis, strokes and lung infarcts.

The spleen may enlarge with initial insults, however becomes nonfunctional. Overtime, the spleen shrinks and develops diffuse calcifications. The ongoing infarcts damage splenic tissue resulting in fibrosis, producing an atrophic, calcified spleen. This processes is referred to as autosplenectomy. On noncontrast CT, the spleen is usually atrophic and diffusely bright in attenuation due to calcifications. A mimicker of diffuse calcification is Thoratrast, giving the spleen a diffuse high-density appearance on noncontrast CT.



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