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

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.

• Authors: David J Lederer, Steven M Kawut, Joshua R Sonett, Efsevia Vakiani, Samuel L Seward Jr, James G White, Jessie S Wilt, Charles C Marboe, William A Gahl, Selim M Arcasoy
• Journal: The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation (J Heart Lung Transplant) Vol. 24 Issue 10 Pg. 1697-9 (Oct 2005) ISSN: 1557-3117 [Electronic] United States
• PMID: 16210149 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

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

Topics

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