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International Journal of Hematology

, Volume 107, Issue 5, pp 610–614 | Cite as

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

  • 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
Case Report

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.

Keywords

Pulmonary alveolar proteinosis Hematopoietic stem cell transplantation Primary immunodeficiency Granulocyte–macrophage colony stimulating factor Bronchoalveolar lavage 

Notes

Acknowledgements

The authors would like to thank Dr. Hisaya Hasegawa in Tokyo Women’s Medical University Medical Center East and Dr. Kazutoshi Cho in Hokkaido University for performing BAL, and Dr. Koh Nakata in Niigata University for measuring anti-GM-CSF antibody.

Compliance with ethical standards

Funding

Funding was supported by Ministry of Education, Culture, Sports, Science, and Technology of Japan, and Ministry of Health, Labour, and Welfare of Japan.

Conflict of interest

The authors declare that there is no conflict of interest.

Supplementary material

12185_2017_2375_MOESM1_ESM.docx (795 kb)
Supplementary material 1 (DOCX 794 kb)

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Copyright information

© The Japanese Society of Hematology 2017

Authors and Affiliations

  • Mari Tanaka-Kubota
    • 1
  • Koji Shinozaki
    • 2
  • Satoshi Miyamoto
    • 1
  • Masakatsu Yanagimachi
    • 1
  • Tsubasa Okano
    • 1
  • Noriko Mitsuiki
    • 1
  • Masahiro Ueki
    • 3
  • Masafumi Yamada
    • 3
  • Kohsuke Imai
    • 4
  • Masatoshi Takagi
    • 4
  • Kazunaga Agematsu
    • 2
    • 5
  • Hirokazu Kanegane
    • 1
  • Tomohiro Morio
    • 1
  1. 1.Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental SciencesTokyo Medical and Dental University (TMDU)TokyoJapan
  2. 2.Department of Pediatrics, School of MedicineShinshu UniversityMatsumotoJapan
  3. 3.Department of PediatricsHokkaido University Graduate School of MedicineSapporoJapan
  4. 4.Department of Community Pediatrics, Perinatal and Maternal Medicine, Graduate School of Medical and Dental SciencesTokyo Medical and Dental University (TMDU)TokyoJapan
  5. 5.Department of Infection and Host Defense, Graduate School of MedicineShinshu UniversityMatsumotoJapan

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