基于 CD56 表达的 NK 大颗粒淋巴细胞白血病分型

NK-large granular lymphocytic leukemia (NK-LGLL) is a rare chronic lymphoproliferative disorder and displays heterogeneity that remains insufficiently defined. CD56 plays a pivotal role in NK-cell maturation linked to cytotoxicity.

However, whether CD56 might be associated with distinctive characteristics in NK-LGLL has not been determined. Hence, this study aims to explore potential associations between CD56 and clinical and biological features in 47 patients with NK-LGLL. Above all, anemia (57. 4%) was the most prevalent symptom. Patients treated with immunosuppressive therapy showed a favorable outcome with 87. 0% achieving remission.

Furthermore, when stratifying patients by CD56 expression on tumor cells, the subset of 28 patients (59. 6%) with diminished CD56 expression was frequently relevant to symptomatic disease (92. 9% vs 15. 8%, P < . 001), comprising anemia (85. 7% vs 15. 8%, P < . 001), neutropenia (67. 9% vs 0. 0%, P < . 001), and splenomegaly (42. 9% vs 10. 5%, P = . 024).

Additionally, this subset demonstrated exclusive STAT3 mutation (61. 9% vs 0. 0%, P = . 003), elevated CD161 levels (54. 5% vs 0. 0%, P < . 001), and bone marrow fibrosis (92. 3% vs 50. 0%, P = 0. 006).

Furthermore, they showed shorter time to first treatment (TTFT) (4-year TTFT: 66. 7% vs 100. 0%, P = . 083) and first-line progression-free survival (PFS) (median PFS: 26. 3 months vs not reached, P = . 112).

Overall, our data indicate that NK-LGLL patients with diminished CD56 expression represent a more aggressive subset compared to those with normal CD56 levels, underscoring the significance of CD56 as a potential prognostic marker and advancing our understanding of the underlying pathogenesis of NK-LGLL.