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

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

Materials And Methods: 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.

Results: 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.

Conclusions: 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.