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

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A Correction to this article was published on 10 October 2018

This article has been updated

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.

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Change history

  • 10 October 2018

    The original version of this article unfortunately contained a mistake in the 7th author’s given name. The correct version is presented above.

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Authors and Affiliations

  1. Division of Hematology and Oncology, Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea

    Eun Sang Yi, Ji Won Lee, Ki Woong Sung, Hong Hoe Koo & Keon Hee Yoo

  2. Department of Pediatrics, Korea University Guro Hospital, Korea University College of Medicine, Seoul, South Korea

    Eun Sang Yi

  3. Department of Pediatrics, Chungbuk National University Hospital, Cheongju, South Korea

    Young Bae Choi

  4. Department of Pediatrics, Cha Bundang Medical Centre, Cha University, Seongnam, South Korea

    Na Hee Lee

  5. Departments of laboratory Medicine & Genetics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea

    Eun-Sook Kang

  6. Division of Pediatric Infectious Diseases and Immunodeficiency, Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea

    Yae-Jean Kim

  7. Department of Health Science and Technology, SAIHST, Sungkyunkwan University, Seoul, South Korea

    Keon Hee Yoo

  8. Stem Cell & Regenerative Medicine Institute, Samsung Medical Center, Seoul, South Korea

    Keon Hee Yoo

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  1. Eun Sang Yi
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  2. Young Bae Choi
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Correspondence to Keon Hee Yoo.

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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.

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Yi, E.S., Choi, Y.B., Lee, N.H. et al. Allogeneic Hematopoietic Cell Transplantation in Patients with Primary Immunodeficiencies in Korea: Eleven-Year Experience in a Single Center. J Clin Immunol 38, 757–766 (2018). https://doi.org/10.1007/s10875-018-0542-7

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  • DOI: https://doi.org/10.1007/s10875-018-0542-7

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