Hematopoietic stem cell transplantation for pulmonary alveolar proteinosis associated with primary immunodeficiency disease.

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.

• Authors: Mari Tanaka-Kubota, Koji Shinozaki, Satoshi Miyamoto, Masakatsu Yanagimachi, Tsubasa Okano, Noriko Mitsuiki, Masahiro Ueki, Masafumi Yamada, Kohsuke Imai, Masatoshi Takagi, Kazunaga Agematsu, Hirokazu Kanegane, Tomohiro Morio
• Journal: International journal of hematology (Int J Hematol) Vol. 107 Issue 5 Pg. 610-614 (May 2018) ISSN: 1865-3774 [Electronic] Japan
• PMID: 29185156 (Publication Type: Case Reports, Journal Article)

Chemical References

• Granulocyte-Macrophage Colony-Stimulating Factor

Topics

• Agammaglobulinemia (etiology)
• Allografts
• Asian People
• Female
• Granulocyte-Macrophage Colony-Stimulating Factor
• Hematopoietic Stem Cell Transplantation
• Humans
• Immunologic Deficiency Syndromes (complications)
• Infant
• Infant, Newborn
• Leukopenia (etiology)
• Monocytes
• Pulmonary Alveolar Proteinosis (etiology, therapy)
• Treatment Outcome