147 Sickle Cell Disease: Acute Chest Syndrome

Clinical Presentation

19-year-old man with known sickle cell anemia and new onset of cough, fever, and chest pain

Radiologic Findings

PA (Fig. 147.1) and lateral (Fig. 147.2) chest radiographs demonstrate moderate cardiomegaly, low lung volumes, and bilateral basilar linear opacities. Note characteristic H-shaped endplate vertebral deformities in the thoracic spine (Fig. 147.2).

Diagnosis

Sickle Cell Disease: Acute Chest Syndrome

Differential Diagnosis

• Bacterial Pneumonia

• Pulmonary Embolism/Infarction

Discussion

Background

Sickle cell anemia is a hemolytic anemia that results from the production of abnormal hemoglobin molecules, which deform the red blood cells and impair their transit through vascular channels. Resultant vascular occlusion produces tissue ischemia and infarction. Acute chest syndrome is the second leading cause of hospitalization in patients with sickle cell anemia.