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  • Title: Diffuse pulmonary fibrosis and the Hermansky-Pudlak syndrome: clinical course and postmortem findings.
    Author: Reynolds SP, Davies BH, Gibbs AR.
    Journal: Thorax; 1994 Jun; 49(6):617-8. PubMed ID: 8016804.
    Abstract:
    The Hermansky-Pudlak syndrome consists of albinism, platelet function defect, pigment laden macrophases and, on occasions, pulmonary fibrosis. The clinical course and postmortem findings of a patient with pulmonary fibrosis which mimicked cryptogenic fibrosing alveolitis are reported. Histological examination revealed a chronic inflammatory infiltrate of pigment laden microphages.
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