Paraneoplastic Pulmonary Alveolar Proteinosis (PPAP) is an acquired form of pulmonary alveolar proteinosis related to an underlying malignancy.
Features of paraneoplastic pulmonary alveolar proteinosis:
(1) presence of an underlying malignancy
(2) onset of pulmonary alveolar proteinosis after development of the malignancy
(3) severity of the pulmonary alveolar proteinosis is parallel to activity of the underlying malignancy (improvement with malignancy control, deterioration with malignancy progression)
Mechanisms:
(1) antibodies to granulocyte-macrophage colony stimulating factor (GM-CSF)
(2) myelodysplasia with macrophages containing mutated GATA2 (GATA binding protein 2)
The PPAP associated with myelodysplasia responds to allogeneic hematopoietic cell transplantation.
To read more or access our algorithms and calculators, please log in or register.
