Large Granular Lymphocytic Leukemia: Clinical Features, Molecular Pathogenesis, Diagnosis and Treatment

• Published in: Cancers Vol. 16; no. 7; p. 1307
• Main Authors: Ullah, Fauzia, Markouli, Mariam, Orland, Mark, Ogbue, Olisaemeka, Dima, Danai, Omar, Najiullah, Mustafa Ali, Moaath K
• Format: Journal Article
• Language: English
• Published: Switzerland MDPI AG 01-04-2024
• Subjects: Anemia; Autoimmune diseases; B cells; Bacterial infections; Classification; Clinical trials; Cloning; Cytokines; Disease; Health aspects; Immunosuppressive agents; Immunotherapy; Infections; Killer cells; Leukemia; Lymphatic leukemia; Lymphocytes; Medical prognosis; Mutation; Natural killer cells; Neutropenia; Neutrophils; NF-κB protein; Pathogenesis; Remission; Stat3 protein; Stat5 protein; Therapeutic targets; Thrombocytopenia; Tumors; Europe; natural killer LGL leukemia; targeted therapies; large granular lymphocytic leukemia; molecular pathogenesis; STAT3
• ISSN: 2072-6694; 2072-6694
• DOI: 10.3390/cancers16071307

Large granular lymphocytic (LGL) leukemia is a lymphoproliferative disorder characterized by persistent clonal expansion of mature T- or natural killer cells in the blood via chronic antigenic stimulation. LGL leukemia is associated with specific immunophenotypic and molecular features, particularly
and
mutations and activation of the
, Fas/Fas-L and
signaling pathways. Disease-related deaths are mainly due to recurrent infections linked to severe neutropenia. The current treatment is based on immunosuppressive therapies, which frequently produce unsatisfactory long-term responses, and for this reason, personalized approaches and targeted therapies are needed. Here, we discuss molecular pathogenesis, clinical presentation, associated autoimmune disorders, and the available treatment options, including emerging therapies.