TY - JOUR
T1 - High risk of relapsed disease in patients with NK/T-cell chronic active Epstein-Barr virus disease outside of Asia
AU - Davila Saldana, Blachy J.
AU - John, Tami
AU - Bonifant, Challice
AU - Buchbinder, David
AU - Chandra, Sharat
AU - Chandrakasan, Shanmuganathan
AU - Chang, Weni
AU - Chen, Leon
AU - Elfassy, Hannah L.
AU - Geerlinks, Ashley V.
AU - Giller, Roger H.
AU - Goyal, Rakesh
AU - Hagin, David
AU - Islam, Shahidul
AU - Mallhi, Kanwaldeep
AU - Miller, Holly K.
AU - Owen, William
AU - Pacheco, Martha
AU - Patel, Niraj C.
AU - Querfeld, Christiane
AU - Quigg, Troy
AU - Richard, Nameeta
AU - Schiff, Deborah
AU - Shereck, Evan
AU - Stenger, Elizabeth
AU - Jordan, Michael B.
AU - Heslop, Helen E.
AU - Bollard, Catherine M.
AU - Cohen, Jeffrey I.
N1 - Publisher Copyright:
© 2022 American Society of Hematology. All rights reserved.
PY - 2022/1/25
Y1 - 2022/1/25
N2 - 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.
AB - 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.
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U2 - 10.1182/bloodadvances.2021005291
DO - 10.1182/bloodadvances.2021005291
M3 - Article
C2 - 34670275
AN - SCOPUS:85123508724
SN - 2473-9529
VL - 6
SP - 452
EP - 459
JO - Blood Advances
JF - Blood Advances
IS - 2
ER -