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

• Type of Material: Article
• Authors: Blachy Dávila J Saldana, George Washington University; Tami John, Baylor College of Medicine; Challice Bonifant, University of Michigan; David Buchbinder, Children's Hospital of Orange County; Sharat Chandra, Cincinnati Children’s Hospital Medical Center; Shanmuganathan Chandrakasan, Emory University; Weni Chang, Brown University; Leon Chen, George Washington University; Hannah L Elfassy, University of Montréal; Ashley Geerlinks, Cincinnati Children’s Hospital Medical Center; Roger H Giller, University of Colorado; Rakesh Goyal, Childrens Mercy Kansas City; David Hagin, University of Tel Aviv; Shahidul Islam, Waikato Hospital; Kanwaldeep Mallhi, University of Washington; Holly K Miller, Phoenix Children’s Hospital; William Owen, Children's Hospital of the King's Daughters; Martha Pacheco, University of Texas Southwestern; Niraj Patel, Emory University; Christiane Querfeld, City of Hope and Beckman Research Institute; Troy Quigg, Methodist Childrens Hosp; Nameeta Richard, Randall Children’s Hospital at Legacy Emanuel; Deborah Schiff, University of California San Diego; Evan Shereck, Oregon Health and Science University; Elizabeth Stenger, Emory University; Michael B Jordan, Cincinnati Children’s Hospital Medical Center; Helen E Heslop, Houston Methodist Hospital; Catherine M Bollard, George Washington University; Jeffrey Cohen, National Institute of Allergy and Infectious Diseases
• Language: English
• Date: 2022-01-25
• Publisher: ELSEVIER
• Publication Version: Final Published Version
• Copyright Statement: © 2022 by The American Society of Hematology.
• License: Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1182/bloodadvances.2021005291
• Title of Journal or Parent Work: BLOOD ADVANCES
• Volume: 6
• Issue: 2
• Start Page: 452
• End Page: 459
• Grant/Funding Information: This work was supported by the intramural research program of the National Institute of Allergy and Infectious Diseases and partially by the National Cancer Institute, National Institutes of Health (5P50CA126752).
• Keywords: PROGNOSTIC-FACTORS; Hematology; LYMPHOMA; NONIMMUNOCOMPROMISED HOSTS; HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS; Science & Technology; Life Sciences & Biomedicine; GUIDELINES
• Research Categories: Health Sciences, Medicine and Surgery; Health Sciences, Immunology

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 5 .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.