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A 37-year-old woman presented with shortness of breath, cough, and fever that began a few days earlier. Her pulse rate was high (110 bpm) with a body temperature of 38.6 °C. Pulmonary auscultation revealed decreased breath sounds over the lower lung fields. Laboratory study showed pronounced leukocytosis (white cell count of 27,500 per cm3) with 89.4% neutrophils and anemia with hemoglobin levels of 9 g per dl. Chest X-ray and computed tomography (CT) scan of the chest revealed bilateral pulmonary opacities over the lower lung fields (Fig. 1 panels A and B). Additionally, the spleen appeared small and heavily calcified (panel C, arrow). Sagittal reconstruction revealed central depression of both superior and inferior end plates of several vertebrae (panel D).
Fig. 1Imaging findings revealed by chest x-ray and computed tomography: bilateral pulmonary opacities (Panels A and B), small and calcified spleen (Panel C) and vertebral end-plate depressions at various heights.
Answer: The patient suffers from sickle cell anemia complicated by acute chest syndrome.
2. Diagnosis
Sickle cell anemia is an autosomal recessive disease with abnormally shaped red blood cells. Disease manifestations are numerous and attributed mostly to anemia, vaso-occlusive events, or superimposed infection [
]. The patient presented with a few of the classical radiological findings associated with sickle cell disease.
The H-shaped deformity (or Lincoln log vertebrae) is considered essentially pathognomonic for sickle cell anemia and results from ischemic changes involving the microvasculature at the end plates [
Hyposplenism is a known feature of sickle cell anemia. In the majority of patients, recurrent vaso-occlusive events ultimately lead to splenic atrophy [
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