Supplementary Case #1

Clinical summary: A 38-y-old man with sickle cell disease (Hb SS) and recent onset of a cold was admitted in December with fever and pain in the extremities, back, and chest. He had had multiple admissions for crises (all culture-negative), paroxysmal atrial fibrillation, and priapism, and had undergone a cholecystectomy (for bilirubin stones), as well as a left hip replacement for avascular necrosis of the femoral head. Physical examination showed: T 40°, BP 94/49 mm Hg, HR 135/min, and oxygen saturation 87% on air. He had diffuse rhonchi in the chest. Laboratory tests showed WBC 6.3 k/µl, Hb 6.7 g/dl, Hct 19%, Plt 62 k/µl, D-dimers >20 mg/L, and fibrinogen 189 mg/dl. Blood was cultured but yielded no growth. Pressors and intravenous fluids were begun. Dyspnea developed, and he died 6 hr after admission. This CT was taken during a stable time, 3 mo before death.

Radiologic-pathologic correlation: The CT (A) shows a wedge-shaped, subpleural lung opacity on the left. On the right, a smaller, subpleural lung opacity (pleural tag) is present anteriorly. Posteriorly, there is accentuation of interlobular septa at the arrows.


The lungs (right 1040 g, left 970 g) showed widespread microvascular congestion and edema. Pneumonia was absent. This slice of right lung (B) shows a subpleural fibrous scar at the base, as well as other pleural indentations and subpleural scars (arrows). Others are also present along the major fissures. These scars are the cause of opacities like those on the CT. The black spot in the middle lobe is a parenchymal lymph node. Vessels and bronchi appear normal, and diffuse parenchymal fibrosis is absent.


Histologically (C), a parenchymal scar, which probably resulted from ischemia (less likely from infection), distorts the architecture. Metaplastic epithelium with goblet cells lines some of the scarred walls, and pale blue mucus fills some air spaces (arrows). Note the indentation of the pleura at the lower left. Many muscular arteries showed intimal thickening (not shown), but no acute thrombi were found.


Two slices of the heart (D) (left ventricle below) show biventricular dilation (right > left) that indicates heart failure. Normally, chambers should be much smaller. The right ventricular wall shows hypertrophy given that it is so dilated. The heart weight was 430 g (normal 325 g). Factors contributing to the hypertrophy and failure include chronic anemia and acute cor pulmonale of thrombotic angiopathy of sickle cell disease.


Diagnosis: Pulmonary thrombotic angiopathy of sickle cell disease (clinical acute chest syndrome). Note: If PFTs and measurement of cardiac pressures had been done, they may have indicated chronic lung disease. See discussion for definitions.


Table of Contents