Successful bilateral lung transplantation for pulmonary fibrosis associated with the Hermansky-Pudlak syndrome.
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.
Lederer
David J
DJ
Division of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, Columbia University College of Physicians and Surgeons, New York, New York 10032, USA.
Kawut
Steven M
SM
Sonett
Joshua R
JR
Vakiani
Efsevia
E
Seward
Samuel L
SL
White
James G
JG
Wilt
Jessie S
JS
Marboe
Charles C
CC
Gahl
William A
WA
Arcasoy
Selim M
SM
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
C102160:HPS1 protein, human; D006490:Hemostatics; D008565:Membrane Proteins; D003894:Deamino Arginine Vasopressin