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

J Thorac Imaging. 2009 May;24(2):115-8. doi: 10.1097/RTI.0b013e3181930ed6.


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.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Adult
  • Female
  • Hematologic Neoplasms / complications*
  • Humans
  • Male
  • Middle Aged
  • Pulmonary Alveolar Proteinosis / diagnostic imaging*
  • Pulmonary Alveolar Proteinosis / etiology*
  • Pulmonary Alveolar Proteinosis / mortality
  • Retrospective Studies
  • Tomography, X-Ray Computed / methods*