Large granular lymphocytic leukemia

LGL-L is a rare disorder characterized by clonal proliferation of cells with large granular lymphocytes. LGL-L can be divided into two major lineage: T(CD3 +) and NK (CD3 -) which have distinct clinical and prognostic features. T-LGL-L represents 80-85% of the all LGL-L. The disease may occur both in men and women, and mostly affects elderly people. Clinical features of T-LGL-L is characterized by recurrent infection related to neutropenia, hepatosplenomegaly and rheumatoid arthritis. Laboratory findings include positive rheumatoid factor and antinuclear antibody. The clinical presentation of NK-LGL leukemia is more acute than T-LGL leukemia, with B symptoms, massive hepatosplenomegaly, involvement of the gastrointestinal tract, and coagulopathy in most cases. Patients are younger and usually have an aggressive course with multiorgan failure and death within a few months of diagnosis despite multiagent chemotherapy. Occasional patients have a chronic NK-LGL leukemia with a more indolent course similar to T-LGL leukemia.