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Abstract
Natural killer (NK) cells are lymphocytes of the native immune system that play a pivotal role in host defense and immune surveillance. While the conceptual view of NK-neoplasms is evolving, little is known about the rare NK lymphoblastic leukemia (NK-LL), which remains as a provisional entity in the 2016 WHO Classification. The goal of this study is to characterize NK-LL cases and compare with other CD56 co-expressing acute leukemias. We identified 105 cases, diagnosed as NK-LL (6), CD56+ acute undifferentiated leukemia (AUL) (6), CD56+T-lymphoblastic leukemia (T-LL) (51), and CD56+ acute myeloid leukemia (AML) (42). Compared to AUL patients, NK-LL patients were significantly younger (p=0.021) and presented with higher white blood cell (WBC) (p=0.037) and platelet counts (p=0.041). Flow cytometry showed more frequent expression of cytoplasmic CD3 (cCD3, p=0.064) and CD33, (p=0.065), while HLA-DR was significantly absent from NK-LL (p=0.035) compared to AUL. Compared to T-ALL, NK-LL cases showed less frequent cCD3 (p=0.002), CD4 (p=0.051), and CD10 expression (p=0.06). The frequency of abnormal karyotypes was similar between NK-LL, AUL, and T-ALL. The mutational profile differed in four leukemia groups, with a significance enrichment of NOTCH1 (p=0.002), ETV6 (p=0.002) and JAK3 (p=0.02) mutations in NK-LL as compared to AML. As compared to T-ALL, NK-LL cases showed a higher number of total mutations (p=0.04) and significantly more frequent ETV6 mutations (p=0.004). Clinical outcome data showed differences in overall survival between all four groups (p=0.0175), but no difference in event free survival (p=0.246). In this largest study to date, we find that that NK-LL shows clinical presentation, immunophenotypic and molecular characteristics distinct from AUL, T-ALL, and AML. Our findings suggest NK-LL is a distinct acute leukemia entity and should be considered in the clinical diagnosis of acute leukemias of ambiguous lineage.
View details for DOI 10.1038/s41379-021-00739-4
View details for PubMedID 33526873