PMID- 37153592
OWN - NLM
STAT- MEDLINE
DCOM- 20230509
LR  - 20230509
IS  - 1664-3224 (Electronic)
IS  - 1664-3224 (Linking)
VI  - 14
DP  - 2023
TI  - The first successful bone marrow transplantation in Vietnam for a young 
      Vietnamese boy with chronic granulomatous disease: a case report.
PG  - 1134852
LID - 10.3389/fimmu.2023.1134852 [doi]
LID - 1134852
AB  - BACKGROUND: Chronic granulomatous disease (CGD) is an inborn error of immunity 
      (IEI) disorder that results from defects in the respiratory burst activity in 
      phagocytes, leading to the inability to kill bacterial and fungal microorganisms. 
      CGD patients usually have a high incidence of morbidity such as infections and 
      autoinflammatory diseases and a high mortality rate. Allogeneic bone marrow 
      transplantation (BMT) is the only definitive cure for patients who suffer from 
      CGD. CASE PRESENTATION: We report the first transplant case of chronic 
      granulomatous disease in Vietnam. A 25-month-old boy with X-linked CGD underwent 
      bone marrow transplantation from his 5-year-old, full-matched human leukocyte 
      antigen (HLA)-carrier sibling after myeloablative conditioning regimen with 
      busulfan 5.1 mg/kg/day for 4 days, fludarabine 30 mg/m(2)/day for 5 days, and 
      rATG (Grafalon-Fresenius) 10 mg/kg/day for 4 days. Neutrophil was engrafted on 
      day 13 posttransplant, donor chimerism was 100% on day 30 with the 
      dihydrorhodamine-1,2,3 (DHR 123) flow cytometric assay test that reached 38% of 
      the normal 45 days posttransplant. Five months after transplant, the patient was 
      free of infection with stable DHR 123 assay at 37%, and donor chimerism remained 
      100%. No sign of a graft-versus-host disease had been observed posttransplant. 
      CONCLUSION: We suggest that bone marrow transplantation is a safe and effectual 
      cure for CGD patients, especially for patients with HLA-identical siblings.
CI  - Copyright (c) 2023 Nguyen-Thanh, Nguyen-Ngoc-Quynh, Dang-Thi, Le-Quynh, 
      Nguyen-Thi-Van, Thuc-Thanh, Dang-Anh, Lee, Cao-Viet and Tran-Minh.
FAU - Nguyen-Thanh, Binh
AU  - Nguyen-Thanh B
AD  - Stem Cells Center, Vietnam National Children's Hospital, Hanoi, Vietnam.
AD  - Pathophysiology and Immunology Department, Hanoi Medical University, Hanoi, 
      Vietnam.
FAU - Nguyen-Ngoc-Quynh, Le
AU  - Nguyen-Ngoc-Quynh L
AD  - Stem Cells Center, Vietnam National Children's Hospital, Hanoi, Vietnam.
FAU - Dang-Thi, Ha
AU  - Dang-Thi H
AD  - Stem Cells Center, Vietnam National Children's Hospital, Hanoi, Vietnam.
FAU - Le-Quynh, Chi
AU  - Le-Quynh C
AD  - Department of Rheumatology, Allergy, and Immunology, Vietnam National Children's 
      Hospital, Hanoi, Vietnam.
FAU - Nguyen-Thi-Van, Anh
AU  - Nguyen-Thi-Van A
AD  - Department of Rheumatology, Allergy, and Immunology, Vietnam National Children's 
      Hospital, Hanoi, Vietnam.
FAU - Thuc-Thanh, Huyen
AU  - Thuc-Thanh H
AD  - Department of Rheumatology, Allergy, and Immunology, Vietnam National Children's 
      Hospital, Hanoi, Vietnam.
FAU - Dang-Anh, Duong
AU  - Dang-Anh D
AD  - Surgical Intensive Care Unit, Vietnam National Children's Hospital, Hanoi, 
      Vietnam.
FAU - Lee, Pamela P
AU  - Lee PP
AD  - Department of Paediatrics and Adolescent Medicine, School of Clinical Medicine, 
      Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong, Hong 
      Kong SAR, China.
FAU - Cao-Viet, Tung
AU  - Cao-Viet T
AD  - Children Heart Center, National Children's Hospital, Hanoi, Vietnam.
FAU - Tran-Minh, Dien
AU  - Tran-Minh D
AD  - Surgical Intensive Care Unit, Vietnam National Children's Hospital, Hanoi, 
      Vietnam.
LA  - eng
PT  - Case Reports
DEP - 20230420
PL  - Switzerland
TA  - Front Immunol
JT  - Frontiers in immunology
JID - 101560960
SB  - IM
MH  - Male
MH  - Humans
MH  - Child, Preschool
MH  - Bone Marrow Transplantation
MH  - *Granulomatous Disease, Chronic/therapy
MH  - Vietnam
MH  - Southeast Asian People
MH  - *Hematopoietic Stem Cell Transplantation/methods
PMC - PMC10156979
OTO - NOTNLM
OT  - Chronic granulomatous disease
OT  - Vietnam
OT  - bone marrow transplant
OT  - case report
OT  - inborn error of immunity
COIS- The authors declare that the research was conducted in the absence of any 
      commercial or financial relationships that could be construed as a potential 
      conflict of interest.
EDAT- 2023/05/08 06:41
MHDA- 2023/05/09 06:42
PMCR- 2023/01/01
CRDT- 2023/05/08 04:17
PHST- 2022/12/31 00:00 [received]
PHST- 2023/04/03 00:00 [accepted]
PHST- 2023/05/09 06:42 [medline]
PHST- 2023/05/08 06:41 [pubmed]
PHST- 2023/05/08 04:17 [entrez]
PHST- 2023/01/01 00:00 [pmc-release]
AID - 10.3389/fimmu.2023.1134852 [doi]
PST - epublish
SO  - Front Immunol. 2023 Apr 20;14:1134852. doi: 10.3389/fimmu.2023.1134852. 
      eCollection 2023.