Aggressive natural killer cell leukemia: is Epstein-Barr virus negativity an indicator of a favorable prognosis?
Aggressive natural killer (NK) cell leukemia (ANKL) is a prototype of an Epstein-Barr virus (EBV)-associated lymphoid malignancy, which is characterized by a fulminant clinical course and a median survival interval <2 months. EBV negativity in ANKL is uncommon, and the characteristics of EBV-negative ANKL are not well defined. We compared the clinicopathological characteristics of EBV-negative ANKL patients (group 1, n = 2) with those of EBV-positive ANKL patients (group 2, n = 14) and reviewed the literature for reports on EBV-negative ANKL cases. EBV-negative and EBV-positive ANKL patients had similar clinical and pathological characteristics, but EBV-negative patients had a longer survival than EBV-positive patients (11.5 vs. 1.5 months, respectively). EBV-negative patients achieved complete remission, but tumors often relapsed after a short interval. In conclusion, EBV-negative ANKL is an uncommon malignancy that pursues a less aggressive clinical course than EBV-positive ANKL.
Ko
Young Hyeh
YH
Department of Pathology, Division of Hematology and Oncology, Samsung Medical Center, Sungkyunkwan University, Seoul, Republic of Korea. yhko310@skku.edu
Park
Sanghui
S
Kim
Kihyun
K
Kim
Seok Jin
SJ
Kim
Won Seog
WS
D000293:Adolescent; D000328:Adult; D000368:Aged; D020031:Epstein-Barr Virus Infections / Q000276:immunology / Q000473:pathology / Q000821:virology*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D007694:Killer Cells, Natural / Q000276:immunology; D015458:Leukemia, T-Cell / Q000276:immunology / Q000473:pathology / Q000821:virology*; D008297:Male; D008875:Middle Aged; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000276:immunology; D012008:Recurrence; D055815:Young Adult