ISSN: 1449-1907International Journal of Medical Sciences
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Int J Med Sci 2023; 20(2):206-210. doi:10.7150/ijms.77583 This issue Cite
Research Paper
Natural Killer Cell Activity Test Helps to Suspect Aggressive Natural Killer Cell Leukemia - Diagnostic Challenge
1. Department of Laboratory Medicine, Inje University College of Medicine, Busan Paik Hospital, Busan, Korea.
2. Department of Laboratory Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
3. Department of Laboratory Medicine, Kyung Hee University School of Medicine and Kyung Hee University Hospital at Gangdong, Seoul, Korea.
4. Department of Hematology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Abstract
Aggressive natural killer cell leukemia (ANKL) is a rare disease with an aggressive clinical course. We aimed to assess the clinicopathological characteristics of the difficult to diagnose ANKL. During ten years, nine patients with ANKL were diagnosed. All the patients exhibited aggressive clinical course and underwent the BM study to rule out lymphoma and hemophagocytic lymphohistiocytosis (HLH). BM examination showed varying degrees of infiltration of neoplastic cells, which were mainly positive for CD2, CD56, cytoplasmic CD3 and EBV in situ hybridization. Five BM aspirates showed histiocytic proliferation with active heomphagocytosis. Normal or increased NK cell activity test results were obtained from 3 patients who were available for testing. Four had multiple BM studies until diagnosis. An aggressive clinical course and positive EBV in situ hybridization, often with associated secondary HLH, should raise the suspicion of an ANKL. Conducting additional supplementary tests such as NK cell activity and NK cell proportion would be helpful for the diagnosis of ANKL.
Keywords: Aggressive natural killer cell leukemia, Epstein-Barr virus, Hemophagocytic lymphohistiocytosis, NK cell activity
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