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  • Title: Successful bilateral lung transplantation for pulmonary fibrosis associated with the Hermansky-Pudlak syndrome.
    Author: Lederer DJ, Kawut SM, Sonett JR, Vakiani E, Seward SL, White JG, Wilt JS, Marboe CC, Gahl WA, Arcasoy SM.
    Journal: J Heart Lung Transplant; 2005 Oct; 24(10):1697-9. PubMed ID: 16210149.
    Abstract:
    Hermansky-Pudlak syndrome (HPS) is a genetic disorder characterized by oculocutaneous albinism, a bleeding diathesis, and in a subset of patients, pulmonary fibrosis. Lung transplantation, the only curative therapy for pulmonary fibrosis, has not been previously reported as a successful treatment strategy for patients with HPS because the bleeding diathesis was thought to contraindicate major thoracic surgery. We successfully performed bilateral sequential lung transplantation in a patient with pulmonary fibrosis and HPS after transfusion of 6 units of platelets. Lung transplantation is a viable therapeutic option in patients with pulmonary fibrosis and only a mild bleeding diathesis associated with HPS.
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