A lung transplant can benefit those whose pulmonary fibrosis (PF) stems from Hermansky-Pudlak syndrome (HPS), a study indicates.
The study, “Clinical management and outcomes of patients with Hermansky-Pudlak syndrome pulmonary fibrosis evaluated for lung transplantation,” was published in the journal PLOS One. HPS is a rare genetic disease characterized by blood platelet dysfunction. Its manifestations include prolonged bleeding, visual impairment, and abnormally light coloring of the skin, hair, and eyes.
Some types of HPS are associated with fibrotic lung diseases. Doctors sometimes recommend a lung transplant for a patient with advanced HPS pulmonary fibrosis. A danger for HPS patients who have surgery like a lung transplant is the potential for excessive bleeding caused by blood platelet defects. Platelets are blood cells that usually promote plotting to stop bleeding.