Treating Rare Lymphoproliferative Malignancies: A Focus on Indolent Large Granular Lymphocytic Leukemia

Abstract

Practice points• Large granular lymphocyte (LGL) leukemias represent approximately 2-3% of all cases of mature lymphocytic leukemias, with equal predilection for men and women, and majority of cases occur in between the fifth to eight decade.• LGL leukemias are proliferations of antigen-activated, cytotoxic T cells; less often, LGL are of natural killer cell lineage, belonging to the innate immune system.• Patients often present with neutropenia and other cytopenias, requiring a broad differential. Approximately 25-33% of the time, autoimmune disorders are associated with LGL leukemia.