Successful bilateral lung transplantation for pulmonary fibrosis associated with the Hermansky-Pudlak syndrome

J Heart Lung Transplant. 2005 Oct;24(10):1697-9. doi: 10.1016/j.healun.2004.11.015.


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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Blood Platelet Disorders / etiology
  • Blood Platelet Disorders / therapy
  • Deamino Arginine Vasopressin / therapeutic use
  • Hemorrhagic Disorders / etiology
  • Hemorrhagic Disorders / prevention & control
  • Hemostatics / therapeutic use
  • Hermanski-Pudlak Syndrome / complications*
  • Hermanski-Pudlak Syndrome / genetics
  • Humans
  • Lung Transplantation*
  • Male
  • Membrane Proteins / genetics
  • Platelet Transfusion
  • Pulmonary Fibrosis / etiology
  • Pulmonary Fibrosis / surgery*
  • Treatment Outcome


  • HPS1 protein, human
  • Hemostatics
  • Membrane Proteins
  • Deamino Arginine Vasopressin