Diagnostic criteria for NK cell large granular lymphocyte leukemia: validation through a multicentric international study.

ABSTRACT:

Abstract

Natural killer (NK) large granular lymphocytic leukemia (LGLL) is a rare lymphoproliferative disorder lacking definitive clonality markers, complicating diagnosis and distinction from reactive NK cell expansions. We previously proposed an NK clonality score with high diagnostic accuracy, but a subset of patients remained unclassified. In this multicenter international study, we refined and validated updated diagnostic criteria using independent training (n = 78) and validation (n = 57) cohorts from 3 national registries (United States, Italy, and France). The revised framework integrates NK score parameters with CC motif chemokine ligand 22 (CCL22) mutations and bone marrow biopsy (BMB) findings. Four major criteria were defined: NK cell count of ≥1.0 × 109/L, killer-cell

SUBMITTER: Pastoret C 

PROVIDER: S-EPMC12870763 | biostudies-literature | 2026 Feb

REPOSITORIES: biostudies-literature