Presentation
Known sickle cell disease, presenting with chest pain.
Patient Data
Age: 40 years
From the case:
Sickle cell disease with acute chest syndrome
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Cardiac silhouette is mildly enlarged. There is bilateral lower zone atelectasis and hazy airspace opacities. Bones are diffusely sclerotic with multi-level H-shaped vertebral bodies. Splenic shadow is difficult to visualize.
Case Discussion
Sickle cell disease is a hemoglobinopathy resulting from a mutation in the beta globin gene. Resultant hemoglobin S, particularly in low oxygen tension states, results in abnormal red blood cell sickling with microvascular occlusion and tissue ischemia/infarction. Given the clinical history in this case, regions of lower zone atelectasis and airspace opacity are supportive of acute chest syndrome.
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