High risk of relapsed disease in patients with NK/T-cell chronic active Epstein-Barr virus disease outside of Asia

Blachy J. Dávila Saldaña, Department of Pediatrics, George Washington University, Washington, DC.
Tami John, Department of Pediatrics, Center for Cell and Gene Therapy, Texas Children's Hospital/Baylor College of Medicine, Houston, TX.
Challice Bonifant, Department of Pediatrics, University of Michigan, Ann Arbor, MI.
David Buchbinder, Department of Hematology, Children's Hospital of Orange County, Orange, CA.
Sharat Chandra, Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
Shanmuganathan Chandrakasan, Aflac Cancer & Blood Disorders Center, Children's Healthcare of Atlanta/Emory University, Atlanta, GA.
Weni Chang, Department of Pediatrics, Hasbro Children's Hospital, The Warren Alpert Medical School of Brown University, Providence, RI.
Leon Chen, Department of Pediatrics, George Washington University, Washington, DC.
Hannah L. Elfassy, Sacrée-Coeur of Montréal Hospital, University of Montréal, Montréal, QC, Canada.
Ashley V. Geerlinks, Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
Roger H. Giller, Pediatric Bone Marrow Transplant & Cellular Therapeutics Program, Children's Hospital Colorado/University of Colorado School of Medicine, Aurora, CO.
Rakesh Goyal, Division of Hematology, Oncology, and Blood and Marrow Transplant, Children's Mercy Kansas City, Kansas City, MO.
David Hagin, Allergy and Clinical Immunology Unit, Department of Medicine, Tel Aviv Sourasky Medical Center and Sackler Faculty of Medicine, University of Tel Aviv, Tel Aviv, Israel.
Shahidul Islam, Department of Haematology, Waikato Hospital, Hamilton, New Zealand.
Kanwaldeep Mallhi, Fred Hutchinson Cancer Research Center, University of Washington, Seattle, WA.
Holly K. Miller, Phoenix Children's Hospital, Phoenix, AZ.
William Owen, Children's Cancer and Blood Disorders Center, Children's Hospital of the King's Daughters, Norfolk, VA.
Martha Pacheco, Pediatric Hematology/Oncology, Children's Health/University of Texas Southwestern, Dallas, TX.
Niraj C. Patel, Levine Children's Hospital, Atrium Health, Charlotte, NC.
Christiane Querfeld, Division of Dermatology and Toni Stephenson Lymphoma Center, City of Hope and Beckman Research Institute, Duarte, CA.
Troy Quigg, Pediatric Blood and Marrow Transplantation Program, Methodist Children's Hospital, San Antonio, TX.
Nameeta Richard, Children's Cancer and Blood Disorders Program, Randall Children's Hospital at Legacy Emanuel, Portland, OR.
Deborah Schiff, Department of Pediatrics, University of California San Diego, La Jolla, CA.
Evan Shereck, Division of Pediatric Hematology/Oncology, Oregon Health and Science University, Portland, OR.
Elizabeth Stenger, Aflac Cancer & Blood Disorders Center, Children's Healthcare of Atlanta/Emory University, Atlanta, GA.
Michael B. Jordan, Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
Helen E. Heslop, Center for Cell and Gene Therapy, Baylor College of Medicine, Houston Methodist Hospital and Texas Children's Hospital, Houston, TX; and.
Catherine M. Bollard, Department of Pediatrics, George Washington University, Washington, DC.
Jeffrey I. Cohen, Laboratory of Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD.

Document Type

Journal Article

Publication Date

1-25-2022

Journal

Blood advances

Volume

6

Issue

2

DOI

10.1182/bloodadvances.2021005291

Abstract

Chronic active Epstein-Barr virus (EBV) disease (CAEBV) is characterized by high levels of EBV predominantly in T and/or natural killer cells with lymphoproliferation, organ failure due to infiltration of tissues with virus-infected cells, hemophagocytic lymphohistiocytosis, and/or lymphoma. The disease is more common in Asia than in the United States and Europe. Although allogeneic hematopoietic stem cell transplantation (HSCT) is considered the only curative therapy for CAEBV, its efficacy and the best treatment modality to reduce disease severity prior to HSCT is unknown. Here, we retrospectively assessed an international cohort of 57 patients outside of Asia. Treatment of the disease varied widely, although most patients ultimately proceeded to HSCT. Though patients undergoing HSCT had better survival than those who did not (55% vs 25%, P < .01), there was still a high rate of death in both groups. Mortality was largely not affected by age, ethnicity, cell-type involvement, or disease complications, but development of lymphoma showed a trend with increased mortality (56% vs 35%, P = .1). The overwhelming majority (75%) of patients who died after HSCT succumbed to relapsed disease. CAEBV remains challenging to treat when advanced disease is present. Outcomes would likely improve with better disease control strategies, earlier referral for HSCT, and close follow-up after HSCT including aggressive management of rising EBV DNA levels in the blood.

Department

Pediatrics

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