A case series of CAEBV of children and young adults treated with reduced‐intensity conditioning and allogeneic bone marrow transplantation: a single‐center study. (28th June 2013)

• Record Type: Journal Article
• Authors: 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
• Is Part Of: European journal of haematology. Volume 91:Number 3(2013:Sep.)
• Page Start: 242
• Page End: 248
• Publication Date: 2013-06-28
• Subjects: Hematology -- Periodicals; Blood -- Diseases -- Periodicals; Blood -- Periodicals; 616.15005
• DOI: 10.1111/ejh.12151
• Languages: English
• ISSNs: 0902-4441

Abstract

<abstract abstract-type="main" id="ejh12151-abs-0001"> <title>Abstract</title> <sec id="ejh12151-sec-0001" sec-type="section"> <title>Background</title> <p>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.</p> </sec> <sec id="ejh12151-sec-0002" sec-type="section"> <title>Patients and Methods</title> <p>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, <italic>in situ</italic> hybridization and microsatellite analysis.</p> </sec> <sec id="ejh12151-sec-0003" sec-type="section"> <title>Results</title> <p>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.</p> </sec> <sec id="ejh12151-sec-0004" sec-type="section"> <title>Conclusions</title> <p>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.</p> </sec> </abstract>