T-Cell Large Granular Lymphocytic Leukemias/Proliferations and Clinical Implications in a Community Practice

Clonal T-Cell large granular lymphocytic leukemias/proliferations were diagnosed in eighteen patients in the community of Kingsport, TN, from 2000–2008, based on peripheral blood and bone marrow morphology, flow cytometry, and T-cell receptor gene rearrangement studies. These clonal expansions were associated with anemia, neutropenia, and thrombocytopenia. Some patients required no treatment, while others responded to weekly methotrexate. Associated disease entities included myelodysplastic syndrome, non-hodgkins lymphoma, multiple myeloma, mast cell disease, febrile illness, and rheumatoid arthritis. Acquired autoimmune hemolytic anemia developed in one case. Anemia was a common presenting feature. Splenomegaly was noted in several cases. Clonal rearrangement of T-cell receptor gamma chain genes was confirmatory in all cases. Chronic disease with uncomplicated cytopenias was noted in the majority. Diagnosis should be suspected in patients with neutropenia and anemia with a relative (and at times an absolute) peripheral blood large granular lymphocytosis. Clonality can be assessed with molecular studies, and therapy is reserved for symptomatic disease or transfusion dependency.