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 front 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 myclogenous leukemia, 1 had myclodysplastic 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 front 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.