Sickle cell disease with acute chest syndrome

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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