Large Granular Lymphocytic Leukemia: From Immunopathogenesis to Treatment of Refractory Disease.

ABSTRACT: Large Granular Lymphocyte Leukemia (LGLL) is a rare, chronic lymphoproliferative disorder of effector cytotoxic T-cells, and less frequently, natural killer (NK) cells. The disease is characterized by an indolent and often asymptomatic course. However, in roughly 50% of cases, treatment is required due to severe transfusion-dependent anemia, severe neutropenia, or moderate neutropenia with associated recurrent infections. LGLL represents an interesting disease process at the intersection of a physiological immune response, autoimmune disorder, and malignant (clonal) proliferation, resulting from the aberrant activation of cellular pathways promoting survival, proliferation, and evasion of apoptotic signaling. LGLL treatment primarily consists of immunosuppressive agents (methotrexate, cycl

SUBMITTER: Zawit M 

PROVIDER: S-EPMC8430581 | biostudies-literature | 2021 Sep

REPOSITORIES: biostudies-literature