Sickle cell disease with acute chest syndrome

Case contributed by Dr Kenny Sim


Known sickle cell disease, presenting with chest pain.

Patient Data

Age: 38
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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Case information

rID: 52505
Published: 10th Apr 2017
Last edited: 10th Apr 2017
System: Haematology
Inclusion in quiz mode: Included

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