PMID- 36461028
OWN - NLM
STAT- MEDLINE
DCOM- 20221206
LR  - 20221213
IS  - 1750-1172 (Electronic)
IS  - 1750-1172 (Linking)
VI  - 17
IP  - 1
DP  - 2022 Dec 2
TI  - Post-transplant cyclophosphamide for GVHD prophylaxis in pediatrics with chronic 
      active Epstein-Barr virus infection after haplo-HSCT.
PG  - 422
LID - 10.1186/s13023-022-02585-2 [doi]
LID - 422
AB  - BACKGROUND: Chronic active Epstein-Barr virus infection (CAEBV) is a rare but 
      life-threatening progressive disease. Human leukocyte antigen 
      (HLA)-haploidentical hematopoietic stem cell transplantation (haplo-HSCT) is the 
      best choice as sometimes HLA-matched donor is not accessible. However, 
      graft-versus-host-disease (GVHD) following transplantation remains a major cause 
      of treatment failure and elevated mortality. Post-transplant cyclophosphamide 
      (PTCy) has recently emerged for effective GVHD prophylaxis in a haploidentical 
      setting in many hematologic malignancies. Here, we report the performance of PTCy 
      for GVHD prophylaxis in a series of CEABV patients treated with haplo-HSCT. 
      METHODS: Consecutive pediatric CAEBV patients who were treated with haplo-HSCT 
      and give PTCy for GVHD prophylaxis were analyzed. 1-year GVHD and relapse-free 
      survival (GRFS), overall survival (OS) and cumulative incidence of 
      moderate-to-severe chronic GVHD (cGVHD) were estimated. RESULTS: A total of 8 
      patients ranging from 2 to 15 years old were included. Among them, 4 patients had 
      early complications after haplo-HSCT. Counts of T-cell subsets increased within 6 
      months post transplantation, indicating an immune reconstitution. Only 1 patient 
      developed grade II acute GVHD, and 2 patients had moderate cGVHD. One patient 
      died from diffuse alveolar hemorrhage within the first year after 
      transplantation. The 1-year GRFS rate, OS rate and cumulative incidence of 
      moderate-to-severe cGVHD were 62.5%, 87.5% and 25.0%, respectively. CONCLUSION: 
      Our findings suggest that, among CAEBV patients treated with haplo-HSCT, PTCy may 
      be an alternative choice for the prevention of GVHD.
CI  - (c) 2022. The Author(s).
FAU - Luo, Rongmu
AU  - Luo R
AD  - Department of Hematology, Aerospace Center Hospital, No. 15, Yuquan Road, Haidian 
      District, Beijing, 100049, China.
FAU - Zhang, Xiaomei
AU  - Zhang X
AD  - Department of Hematology, Aerospace Center Hospital, No. 15, Yuquan Road, Haidian 
      District, Beijing, 100049, China.
FAU - Wang, Ya
AU  - Wang Y
AD  - Department of Hematology, Senior Department of Pediatrics, The Seventh Medical 
      Center of PLA General Hospital, Beijing, 100700, China.
FAU - Man, Qihang
AU  - Man Q
AD  - Department of Hematology, Aerospace Center Hospital, No. 15, Yuquan Road, Haidian 
      District, Beijing, 100049, China.
FAU - Gu, Wenjing
AU  - Gu W
AD  - Department of Hematology, Aerospace Center Hospital, No. 15, Yuquan Road, Haidian 
      District, Beijing, 100049, China.
FAU - Tian, Zhengqin
AU  - Tian Z
AD  - Department of Hematology, Aerospace Center Hospital, No. 15, Yuquan Road, Haidian 
      District, Beijing, 100049, China.
FAU - Wang, Jingbo
AU  - Wang J
AUID- ORCID: 0000-0003-3183-8546
AD  - Department of Hematology, Aerospace Center Hospital, No. 15, Yuquan Road, Haidian 
      District, Beijing, 100049, China. wangjingbo721@126.com.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20221202
PL  - England
TA  - Orphanet J Rare Dis
JT  - Orphanet journal of rare diseases
JID - 101266602
RN  - 8N3DW7272P (Cyclophosphamide)
SB  - IM
MH  - Child
MH  - Humans
MH  - Child, Preschool
MH  - Adolescent
MH  - *Epstein-Barr Virus Infections/prevention & control
MH  - *Graft vs Host Disease/drug therapy/prevention & control
MH  - Herpesvirus 4, Human
MH  - *Hematopoietic Stem Cell Transplantation
MH  - Cyclophosphamide/therapeutic use
MH  - Persistent Infection
PMC - PMC9716678
OTO - NOTNLM
OT  - Anti-thymocyte globulin
OT  - Chronic active Epstein-Barr virus infection
OT  - Graft-versus-host disease prophylaxis
OT  - Hematopoietic stem cell transplantation
OT  - Post-transplant cyclophosphamide
COIS- The authors declare that they have no competing interests.
EDAT- 2022/12/03 06:00
MHDA- 2022/12/07 06:00
PMCR- 2022/12/02
CRDT- 2022/12/02 23:48
PHST- 2022/01/06 00:00 [received]
PHST- 2022/11/22 00:00 [accepted]
PHST- 2022/12/02 23:48 [entrez]
PHST- 2022/12/03 06:00 [pubmed]
PHST- 2022/12/07 06:00 [medline]
PHST- 2022/12/02 00:00 [pmc-release]
AID - 10.1186/s13023-022-02585-2 [pii]
AID - 2585 [pii]
AID - 10.1186/s13023-022-02585-2 [doi]
PST - epublish
SO  - Orphanet J Rare Dis. 2022 Dec 2;17(1):422. doi: 10.1186/s13023-022-02585-2.