T-cell large granular leukemia and related proliferations

Session 9 of the 2005 Society for Hematopathology/European Association for Haematopathology Workshop focused on large granular lymphocyte (LGL) leukemias and related disorders. T-cell LGL (T-LGL) leukemias, discussed herein, account for 2% to 3% of cases of small lymphocytic leukemia. T-LGL diseases cover a heterogeneous spectrum of disorders that include reactive conditions, typically associated with autoimmune disease, to outright leukemia. These disorders are found in older people, with an average age at initial examination of approximately 60 years and a median survival of more than 10 years in T-LGL leukemia. Systemic symptoms and neutropenia are common at initial examination. Lymphocytosis, composed of small mature lymphocytes with increased cytoplasm, is common. The spleen and bone marrow are involved in T-LGL leukemia, although morphologic findings may be subtle. The immunophenotype is typically that of CD3+/CD8+ cytotoxic T cells. Some cases may be due to chronic immune stimulation, with subsequent clonal escape and proliferation of a neoplastic population of lymphocytes.