Perspective
Hereditary pulmonary alveolar proteinosis: Allogeneic Hematopoietic Stem Cell Transplantation Following Lung Transplantation
Author(s): Martin Amis*
A rare, widespread lung condition known as Pulmonary Alveolar Proteinosis (PAP) is characterised by surfactant buildup in the small airways as a result of suboptimal alveolar macrophage clearance, impairing gas exchange. The current gold standard of care for PAP is whole lung lavage. When whole lung lavage or other experimental therapy approaches are inadequate or in cases of severe pulmonary fibrosis brought on by PAP, lung transplantation is a recognised alternative for treatment. Recurrence of PAP in the transplanted lungs, especially in hereditary PAP, is a drawback of lung transplantation. The Granulocyte Macrophage-Colony Stimulating Factor (GM-CSF) receptor genes are the source of the genetic abnormalities that lead to the hereditary form of PAP, an extremely rare illness that directly affects bone marrow-derived monocytes that later develop into macrophages in the lung. As a r.. Read More»
DOI:
10.37532/puljcgg.22.5(3) 1-2
