Neutropenia and Large Granular Lymphocyte Leukemia: From Pathogenesis to Therapeutic Options.

ABSTRACT: Large granular lymphocyte leukemia (LGLL) is a rare lymphoproliferative disorder characterized by the clonal expansion of cytotoxic T-LGL or NK cells. Chronic isolated neutropenia represents the clinical hallmark of the disease, being present in up to 80% of cases. New advances were made in the biological characterization of neutropenia in these patients, in particular STAT3 mutations and a discrete immunophenotype are now recognized as relevant features. Nevertheless, the etiology of LGLL-related neutropenia is not completely elucidated and several mechanisms, including humoral abnormalities, bone marrow infiltration/substitution and cell-mediated cytotoxicity might cooperate to its pathogenesis. As a consequence of the multifactorial nature of LGLL-related neutropenia, a targeted therape

SUBMITTER: Calabretto G 

PROVIDER: S-EPMC8534439 | biostudies-literature | 2021 Oct

REPOSITORIES: biostudies-literature