Abstract
Large granular lymphocyte (LGL) leukemia is a lymphoproliferative disorder of cytotoxic cells. T-cell LGL (T-LGL) leukemia is characterized by accumulation of cytotoxic T cells in blood and infiltration of the bone marrow, liver or spleen. Population-based studies have not been reported in LGL leukemia. We present clinical characteristics, natural history and risk factors for poor survival in patients with LGL leukemia using the Surveillance, Epidemiology, and End Results Program (SEER) and the United States National Cancer Data Base (NCDB). LGL leukemia is an extremely rare disease with the incidence of 0.2 cases per 1 000 000 individuals. The median age at diagnosis was 66.5 years with females likely to be diagnosed at 3 years earlier compared with males. Analysis of patient-level data using NCDB (n=978) showed that 45% patients with T-LGL leukemia required some form of systemic treatment at the time of diagnosis. T-LGL leukemia patients have reduced survival compared with general population, with a median overall survival of 9 years. Multivariate analysis showed that age >60 years at the time of diagnosis and the presence of significant comorbidities were independent predictors of poor survival.
Highlights
Large granular lymphocyte (LGL) leukemia is a chronic lymphoproliferative disorder of cytotoxic T or natural killer cells
Using the National Cancer Data Base (NCDB) database, patient-level data was available for 1150 patients with LGL leukemia
The incidence of T-cell LGL (T-LGL) leukemia was similar between the sexes with a male-tofemale ratio of 1.05
Summary
Large granular lymphocyte (LGL) leukemia is a chronic lymphoproliferative disorder of cytotoxic T or natural killer cells. T-cell LGL (T-LGL) leukemia is characterized by clonal proliferation of CD3+/CD8+ cytotoxic T lymphocytes and infiltration of the bone marrow, liver or spleen. The diagnosis of T-LGL leukemia is based on peripheral blood expansion of the leukemic LGL population (40.5 × 109/l) in association with CD3+/CD8+ phenotype and evidence of clonality as suggested by clonal rearrangement of the T-cell receptor gene using PCR or flow cytometry.[1,2,3]. T-LGL leukemia is a rare disease representing ~ 2–5% of chronic lymphoproliferative diseases in the US.[3] Despite significant advances in understanding biology and therapeutics of LGL leukemia, the incidence, demographics, natural history, treatments and long-term prognosis of the disease remain poorly characterized
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