PMID- 35438460 OWN - NLM STAT- MEDLINE DCOM- 20220929 LR - 20220929 IS - 1865-3774 (Electronic) IS - 0925-5710 (Linking) VI - 116 IP - 4 DP - 2022 Oct TI - Successful HLA-haploidentical stem cell transplantation with post-transplant cyclophosphamide in an older patient with chronic active Epstein-Barr virus infection. PG - 630-634 LID - 10.1007/s12185-022-03345-5 [doi] AB - Chronic active Epstein-Barr virus infection (CAEBV) is a subtype of EBV-associated T/NK cell lymphoproliferative disease and is only curable by allogeneic hematopoietic stem cell transplantation. However, finding a human leukocyte antigen (HLA)-matched donor at a suitable time can sometimes be difficult. We report the case of a 60-year-old woman who received prednisolone (PSL) after being diagnosed with autoimmune hepatitis 3 years earlier. She suddenly developed high fever and impaired liver function. Based on a high EBV DNA load in the peripheral blood, CAEBV was diagnosed. The patient was started on cooling therapy with PSL, cyclosporine, and etoposide, which reduced symptoms. Subsequently, she received HLA-haploidentical stem cell transplantation (haplo-SCT) with reduced-intensity conditioning (fludarabine 25 mg/m(2) for 5 days, melphalan 50 mg/m(2) for 2 days, and total body irradiation at 2 Gy) and post-transplant cyclophosphamide (PTCy) because she lacked an HLA-matched donor. Liver function was restored, and EBV DNA load in peripheral white blood cells became undetectable. The patient is alive without relapse or severe complications over 1 year after transplantation. To our knowledge, this is the first report of successful haplo-SCT with PTCy for CAEBV. This approach may be an alternative therapeutic option for CAEBV patients lacking an HLA-matched donor. CI - (c) 2022. Japanese Society of Hematology. FAU - Maegaki, Masaya AU - Maegaki M AD - Department of Hematology, Tottori University Hospital, 36-1 Nishi-cho, Yonago, Tottori, 683-8504, Japan. FAU - Kawamura, Koji AU - Kawamura K AD - Department of Hematology, Tottori University Hospital, 36-1 Nishi-cho, Yonago, Tottori, 683-8504, Japan. AD - Division of Clinical Laboratory Medicine, Department of Multidisciplinary Internal Medicine, Faculty of Medicine, Tottori University, Tottori, Japan. FAU - Hara, Kentaro AU - Hara K AD - Department of Hematology, Tottori University Hospital, 36-1 Nishi-cho, Yonago, Tottori, 683-8504, Japan. FAU - Hosoda, Rina AU - Hosoda R AD - Department of Hematology, Tottori University Hospital, 36-1 Nishi-cho, Yonago, Tottori, 683-8504, Japan. FAU - Suzuki, Sayaka AU - Suzuki S AD - Department of Hematology, Tottori University Hospital, 36-1 Nishi-cho, Yonago, Tottori, 683-8504, Japan. AD - Division of Clinical Laboratory Medicine, Department of Multidisciplinary Internal Medicine, Faculty of Medicine, Tottori University, Tottori, Japan. FAU - Hosoda, Yuzuru AU - Hosoda Y AD - Department of Hematology, Tottori University Hospital, 36-1 Nishi-cho, Yonago, Tottori, 683-8504, Japan. AD - Division of Clinical Laboratory Medicine, Department of Multidisciplinary Internal Medicine, Faculty of Medicine, Tottori University, Tottori, Japan. FAU - Imadome, Ken-Ichi AU - Imadome KI AD - Department of Advanced Medicine for Infections, National Center for Child Health and Development (NCCHD), Tokyo, Japan. FAU - Motokura, Toru AU - Motokura T AD - Division of Clinical Laboratory Medicine, Department of Multidisciplinary Internal Medicine, Faculty of Medicine, Tottori University, Tottori, Japan. FAU - Fukuda, Tetsuya AU - Fukuda T AUID- ORCID: 0000-0002-4632-8429 AD - Department of Hematology, Tottori University Hospital, 36-1 Nishi-cho, Yonago, Tottori, 683-8504, Japan. tfukuda@tottori-u.ac.jp. LA - eng PT - Case Reports PT - Journal Article DEP - 20220418 PL - Japan TA - Int J Hematol JT - International journal of hematology JID - 9111627 RN - 0 (Cyclosporins) RN - 0 (HLA Antigens) RN - 6PLQ3CP4P3 (Etoposide) RN - 8N3DW7272P (Cyclophosphamide) RN - 9PHQ9Y1OLM (Prednisolone) RN - Q41OR9510P (Melphalan) SB - IM MH - Cyclophosphamide/therapeutic use MH - *Cyclosporins/therapeutic use MH - *Epstein-Barr Virus Infections/complications MH - Etoposide/therapeutic use MH - Female MH - *Graft vs Host Disease/etiology MH - HLA Antigens MH - *Hematopoietic Stem Cell Transplantation/adverse effects MH - Herpesvirus 4, Human MH - Humans MH - Melphalan/therapeutic use MH - Middle Aged MH - Prednisolone/therapeutic use MH - Transplantation Conditioning/adverse effects OTO - NOTNLM OT - Chronic active Epstein-Barr virus infection (CAEBV) OT - HLA-haploidentical stem cell transplantation (haplo-SCT) OT - Post-transplant cyclophosphamide (PTCy) EDAT- 2022/04/20 06:00 MHDA- 2022/09/30 06:00 CRDT- 2022/04/19 12:10 PHST- 2021/12/19 00:00 [received] PHST- 2022/03/31 00:00 [accepted] PHST- 2022/03/31 00:00 [revised] PHST- 2022/04/20 06:00 [pubmed] PHST- 2022/09/30 06:00 [medline] PHST- 2022/04/19 12:10 [entrez] AID - 10.1007/s12185-022-03345-5 [pii] AID - 10.1007/s12185-022-03345-5 [doi] PST - ppublish SO - Int J Hematol. 2022 Oct;116(4):630-634. doi: 10.1007/s12185-022-03345-5. Epub 2022 Apr 18.