AI Article Synopsis
- - Pulmonary alveolar proteinosis (PAP) is a rare lung disorder that results in a buildup of surfactant-like substances in the alveoli, causing severe breathing issues.
- - The report discusses two Japanese infants suffering from PAP alongside low levels of immunoglobulins (hypogammaglobulinemia) and a decrease in white blood cells (monocytopenia), suggesting they might have a primary immunodeficiency (PID).
- - Both infants were effectively treated with allogeneic hematopoietic stem cell transplantation (HSCT), indicating that this treatment could potentially cure PAP related to immunodeficiency.
Article Abstract
Pulmonary alveolar proteinosis (PAP) is a rare disorder that is characterized by the excessive accumulation of surfactant-like materials in the alveoli, leading to hypoxemic respiratory failure. We describe two Japanese infants with PAP associated with hypogammaglobulinemia and monocytopenia. These patients may have underlying primary immunodeficiency (PID) and were successfully treated with allogeneic hematopoietic stem cell transplantation (HSCT). This report indicates that allogeneic HSCT may provide a curative treatment for PAP associated with PID.
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| http://dx.doi.org/10.1007/s12185-017-2375-1 | DOI Listing |
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