title: 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.affiliation: Division of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, Columbia University College of Physicians and Surgeons, New York, New York 10032, USA.
authors.forename: David J
authors.initials: DJ
authors.lastname: Lederer
authors.forename: Steven M
authors.initials: SM
authors.lastname: Kawut
authors.forename: Joshua R
authors.initials: JR
authors.lastname: Sonett
authors.forename: Efsevia
authors.initials: E
authors.lastname: Vakiani
authors.forename: Samuel L
authors.initials: SL
authors.lastname: Seward
authors.forename: James G
authors.initials: JG
authors.lastname: White
authors.forename: Jessie S
authors.initials: JS
authors.lastname: Wilt
authors.forename: Charles C
authors.initials: CC
authors.lastname: Marboe
authors.forename: William A
authors.initials: WA
authors.lastname: Gahl
authors.forename: Selim M
authors.initials: SM
authors.lastname: Arcasoy
mesh_terms: D000328:Adult; D001791:Blood Platelet Disorders / Q000209:etiology / Q000628:therapy; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D006474:Hemorrhagic Disorders / Q000209:etiology / Q000517:prevention & control; D006490:Hemostatics / Q000627:therapeutic use; D022861:Hermanski-Pudlak Syndrome / Q000150:complications* / Q000235:genetics; D006801:Humans; D016040:Lung Transplantation; D008297:Male; D008565:Membrane Proteins / Q000235:genetics; D017713:Platelet Transfusion; D011658:Pulmonary Fibrosis / Q000209:etiology / Q000601:surgery*; D016896:Treatment Outcome
chemical_list: C102160:HPS1 protein, human; D006490:Hemostatics; D008565:Membrane Proteins; D003894:Deamino Arginine Vasopressin