Secondary pulmonary alveolar proteinosis: a confusing and potentially serious complication of hematologic malignancy

We analyzed the computed tomography and clinical findings of pulmonary alveolar proteinosis secondary to hematologic malignancy.

Seven patients with hematologic malignancy and pathologically proven secondary pulmonary alveolar proteinosis were identified from 2000 to 2007. Six had chest computed tomography scans, which were analyzed retrospectively; medical records were also reviewed.

Patient age ranged from 30 to 54 years. Four had chronic myelogenous leukemia, 1 had myelodysplastic syndrome, and 1 had cutaneous T-cell lymphoma. As in idiopathic pulmonary alveolar proteinosis, geographic ground-glass opacities with or without septal thickening were most common (5/6). No axial or zonal predominance was present. Two patients died from respiratory failure.

It is important to consider secondary pulmonary alveolar proteinosis as a cause of geographic ground-glass opacities and septal thickening in a patient with hematologic malignancy. Whereas idiopathic pulmonary alveolar proteinosis has a low mortality rate, the death of 2 of our 6 patients implies that secondary pulmonary alveolar proteinosis may have a worse prognosis. Our case of secondary pulmonary alveolar proteinosis associated with cutaneous T-cell lymphoma is the first described in the literature.