International Journal of Hematology

DOI: 10.1007/s12185-017-2375-1 Pages: 1-5

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

1. Tokyo Medical and Dental University (TMDU), Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences

2. Shinshu University, Department of Pediatrics, School of Medicine

3. Hokkaido University Graduate School of Medicine, Department of Pediatrics

4. Tokyo Medical and Dental University (TMDU), Department of Community Pediatrics, Perinatal and Maternal Medicine, Graduate School of Medical and Dental Sciences

5. Shinshu University, Department of Infection and Host Defense, Graduate School of Medicine

Correspondence to:
Hirokazu Kanegane
Email: hkanegane.ped@tmd.ac.jp

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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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