Diffuse pulmonary fibrosis and the Hermansky-Pudlak syndrome: clinical course and postmortem findings.
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.
Asthma and Allergy Unit, Sully Hospital, Penarth, South Glamorgan.
S P
SP
Reynolds
B H
BH
Davies
A R
AR
Gibbs
D000328:Adult; D016115:Albinism, Oculocutaneous / Q000150:complications* / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology; D005260:Female; D006801:Humans; D008062:Lipofuscin / Q000032:analysis; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology; D008264:Macrophages / Q000737:chemistry / Q000473:pathology; D011658:Pulmonary Fibrosis / Q000150:complications* / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology; D011859:Radiography
D008062:Lipofuscin