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Journal of Clinical Immunology

, Volume 38, Issue 7, pp 757–766 | Cite as

Allogeneic Hematopoietic Cell Transplantation in Patients with Primary Immunodeficiencies in Korea: Eleven-Year Experience in a Single Center

  • Eun Sang Yi
  • Young Bae Choi
  • Na Hee Lee
  • Ji Won Lee
  • Ki Woong Sung
  • Hong Hoe Koo
  • Eun-Sook Kang
  • Yae-Jean Kim
  • Keon Hee Yoo
Original Article

Abstract

Purpose

We aimed to report our single-center experience of allogeneic hematopoietic cell transplantation (HCT), which has been the only curative option for certain patients with lethal primary immunodeficiencies (PIDs).

Methods

We summarized the results of HCT performed for patients with PIDs for 11 consecutive years from 2006 to 2016 at Samsung Medical Center, Seoul, Korea. Twenty-six patients with PIDs received HCT. Most had chronic granulomatous disease (42.3%), Wiskott Aldrich syndrome (15.4%), or severe combined immunodeficiency (11.5%).

Results

Nine patients (34.6%) received HCT during the former half period and 17 patients (65.4%) during the latter half period. Donor types were categorized as: matched sibling donor (n = 5), unrelated donor (n = 17), and familial mismatched donor (FMMD) (n = 4). Unrelated HCT and FMMD transplantation were increasingly performed in the latter half period compared to the first (5 vs. 16, P = 0.034). Five patients experienced initial engraftment failure, but all of them were eventually engrafted after additional HCTs. The 3-year probability of overall survival was 72.0%. Seven patients (26.9%) died, and the causes of death were bacterial sepsis (n = 4), pneumonia (n = 1), chronic graft-versus-host disease (GVHD) (n = 1), and diffuse alveolar hemorrhage (n = 1). Two patients with bacterial sepsis and a patient with pneumonia also had chronic GVHD. Unrelated HCT and use of methotrexate were associated with poor outcome. Complete chimerism was attained in 85.0% at 1 year after HCT.

Conclusion

PID candidates have been increasingly identified for allogeneic HCT in Korea, and the majority of them could be cured by HCT. Establishment of a systematic registry of PID patients for HCT is needed.

Keywords

Hematopoietic cell transplantation bone marrow transplantation primary immunodeficiency child 

Notes

Compliance with Ethical Standards

Conflicts of Interest

The authors declare that there are no conflicts of interest.

Ethical Standards

The study was approved by the Institutional Review Board of Samsung Medical Center and performed in accordance with the principles of the Declaration of Helsinki. The informed consent was waived.

Supplementary material

10875_2018_542_MOESM1_ESM.docx (22 kb)
Supplementary Table S1 (DOCX 22 kb)

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Eun Sang Yi
    • 1
    • 2
  • Young Bae Choi
    • 3
  • Na Hee Lee
    • 4
  • Ji Won Lee
    • 1
  • Ki Woong Sung
    • 1
  • Hong Hoe Koo
    • 1
  • Eun-Sook Kang
    • 5
  • Yae-Jean Kim
    • 6
  • Keon Hee Yoo
    • 1
    • 7
    • 8
  1. 1.Division of Hematology and Oncology, Department of Pediatrics, Samsung Medical CenterSungkyunkwan University School of MedicineSeoulSouth Korea
  2. 2.Department of PediatricsKorea University Guro Hospital, Korea University College of MedicineSeoulSouth Korea
  3. 3.Department of PediatricsChungbuk National University HospitalCheongjuSouth Korea
  4. 4.Department of Pediatrics, Cha Bundang Medical CentreCha UniversitySeongnamSouth Korea
  5. 5.Departments of laboratory Medicine & Genetics, Samsung Medical CenterSungkyunkwan University School of MedicineSeoulSouth Korea
  6. 6.Division of Pediatric Infectious Diseases and Immunodeficiency, Department of Pediatrics, Samsung Medical CenterSungkyunkwan University School of MedicineSeoulSouth Korea
  7. 7.Department of Health Science and TechnologySAIHST, Sungkyunkwan UniversitySeoulSouth Korea
  8. 8.Stem Cell & Regenerative Medicine InstituteSamsung Medical CenterSeoulSouth Korea

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