Post-transplant cyclophosphamide for GVHD prophylaxis in pediatrics with chronic active Epstein-Barr virus infection after haplo-HSCT
Abstract 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,...
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BMC
2022-12-01
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Series: | Orphanet Journal of Rare Diseases |
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Online Access: | https://doi.org/10.1186/s13023-022-02585-2 |
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author | Rongmu Luo Xiaomei Zhang Ya Wang Qihang Man Wenjing Gu Zhengqin Tian Jingbo Wang |
author_facet | Rongmu Luo Xiaomei Zhang Ya Wang Qihang Man Wenjing Gu Zhengqin Tian Jingbo Wang |
author_sort | Rongmu Luo |
collection | DOAJ |
description | Abstract 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. |
first_indexed | 2024-04-13T10:06:50Z |
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language | English |
last_indexed | 2024-04-13T10:06:50Z |
publishDate | 2022-12-01 |
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series | Orphanet Journal of Rare Diseases |
spelling | doaj.art-d0f8f5bf9aab4ce4a46b8e3e0036bb932022-12-22T02:51:04ZengBMCOrphanet Journal of Rare Diseases1750-11722022-12-011711910.1186/s13023-022-02585-2Post-transplant cyclophosphamide for GVHD prophylaxis in pediatrics with chronic active Epstein-Barr virus infection after haplo-HSCTRongmu Luo0Xiaomei Zhang1Ya Wang2Qihang Man3Wenjing Gu4Zhengqin Tian5Jingbo Wang6Department of Hematology, Aerospace Center HospitalDepartment of Hematology, Aerospace Center HospitalDepartment of Hematology, Senior Department of Pediatrics, The Seventh Medical Center of PLA General HospitalDepartment of Hematology, Aerospace Center HospitalDepartment of Hematology, Aerospace Center HospitalDepartment of Hematology, Aerospace Center HospitalDepartment of Hematology, Aerospace Center HospitalAbstract 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.https://doi.org/10.1186/s13023-022-02585-2Chronic active Epstein-Barr virus infectionHematopoietic stem cell transplantationAnti-thymocyte globulinPost-transplant cyclophosphamideGraft-versus-host disease prophylaxis |
spellingShingle | Rongmu Luo Xiaomei Zhang Ya Wang Qihang Man Wenjing Gu Zhengqin Tian Jingbo Wang Post-transplant cyclophosphamide for GVHD prophylaxis in pediatrics with chronic active Epstein-Barr virus infection after haplo-HSCT Orphanet Journal of Rare Diseases Chronic active Epstein-Barr virus infection Hematopoietic stem cell transplantation Anti-thymocyte globulin Post-transplant cyclophosphamide Graft-versus-host disease prophylaxis |
title | Post-transplant cyclophosphamide for GVHD prophylaxis in pediatrics with chronic active Epstein-Barr virus infection after haplo-HSCT |
title_full | Post-transplant cyclophosphamide for GVHD prophylaxis in pediatrics with chronic active Epstein-Barr virus infection after haplo-HSCT |
title_fullStr | Post-transplant cyclophosphamide for GVHD prophylaxis in pediatrics with chronic active Epstein-Barr virus infection after haplo-HSCT |
title_full_unstemmed | Post-transplant cyclophosphamide for GVHD prophylaxis in pediatrics with chronic active Epstein-Barr virus infection after haplo-HSCT |
title_short | Post-transplant cyclophosphamide for GVHD prophylaxis in pediatrics with chronic active Epstein-Barr virus infection after haplo-HSCT |
title_sort | post transplant cyclophosphamide for gvhd prophylaxis in pediatrics with chronic active epstein barr virus infection after haplo hsct |
topic | Chronic active Epstein-Barr virus infection Hematopoietic stem cell transplantation Anti-thymocyte globulin Post-transplant cyclophosphamide Graft-versus-host disease prophylaxis |
url | https://doi.org/10.1186/s13023-022-02585-2 |
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