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A case series of CAEBV of children and young adults treated with reduced‐intensity conditioning and allogeneic bone marrow transplantation: a single‐center study
Author(s) -
Watanabe Yuko,
Sasahara Yoji,
Satoh Miki,
Looi Chung Yeng,
Katayama Saori,
Suzuki Tasuku,
Suzuki Nobu,
Ouchi Meri,
Horino Satoshi,
Moriya Kunihiko,
Nanjyo Yuka,
Onuma Masaei,
Kitazawa Hiroshi,
Irie Masahiro,
Niizuma Hidetaka,
Uchiyama Toru,
Rikiishi Takeshi,
Kumaki Satoru,
Minegishi Masayoshi,
Wada Taizo,
Yachie Akihiro,
Tsuchiya Shigeru,
Kure Shigeo
Publication year - 2013
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/ejh.12151
Subject(s) - fludarabine , hematopoietic stem cell transplantation , transplantation , total body irradiation , cyclophosphamide , immunology , medicine , chemotherapy , bone marrow , regimen , gastroenterology , oncology
Background Epstein–Barr virus ( EBV )‐infected T or NK cells cause chronic active EBV infection ( CAEBV ). Allogeneic hematopoietic stem cell transplantation ( HSCT ) is curative treatment for CAEBV patients. However, chemotherapy prior to HSCT and optimal conditioning regimen for allogeneic HSCT are still controversial. Patients and Methods We retrospectively analyzed five patients with CAEBV treated with reduced‐intensity conditioning ( RIC ) consisted of fludarabine, cyclophosphamide, and low‐dose total‐body irradiation followed by allogeneic bone marrow transplantation in a single institute. Only one of five patients received chemotherapy prior to transplantation. We analyzed EBV ‐infected cells in a patient whose EBV load increased after HSCT by T‐cell repertoire assay, separation of T‐cell subpopulations, in situ hybridization and microsatellite analysis. Results All five patients achieved engraftment, complete chimera, and eradication of EBV load. All patients have been alive without any serious regimen‐related toxicity for more than 16 months following HSCT . However, one patient transplanted from HLA ‐matched sibling donor developed clonal proliferation of CD4+ Vβ3+ T cells caused by monoclonal EBV infection on day 99 after transplantation. Further analysis revealed that the CD4+ Vβ3+ T cells selectively harbored EBV genome, and these infected cells were derived from donor T cells. Conclusions Allogeneic HSCT with RIC is a safe and effective treatment for better overall survival and less regimen‐related toxicity in patients with CAEBV . Our first pediatric case reported in the literature suggests that we should consider the possibility of persistent EBV infection in donor T cells as well as the relapse in recipient cells if EBV load increases after allogeneic HSCT .