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Research Article
Diffuse pulmonary fibrosis and the Hermansky-Pudlak syndrome: clinical course and postmortem findings.
  1. S P Reynolds,
  2. B H Davies,
  3. A R Gibbs
  1. Asthma and Allergy Unit, Sully Hospital, Penarth, South Glamorgan.

    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.

    https://doi.org/10.1136/thx.49.6.617

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