Large granular lymphocyte (LGL) leukemia

A clonal proliferation disorder of cytotoxic T cells associated with pancytopenia and modest lymphocytosis.

It is a lymphoproliferation of mature cytotoxic T cells or natural (NK) cells.

Characterized by pancytopenia, which contributes the majority of its morbidity.

Generally affects elderly patients.

One third of patients have associated rheumatoid arthritis.

Often associated with an underlying or autoimmune disease, or malignancy, including hematologic disorders and solid tumors.

Approximately 1/3 patients are asymptomatic at presentation.

Symptoms associated include: neutropenia with recurrent infections, splenomegaly, and B symptoms such as fatigue, weight loss, and night sweats.

Invasion of large granular lymphocytes in the bone marrow, spleen and liver.

May be derived from either CD3- or CD+ large granular lymphocytes, designated NK and CD3+ large granular lymphocyte leukemia.

90% of patients with LGL/rheumatoid arthritis have DR4 haplotype, as do almost all patients with Felty’s syndrome.

T cell form typified by neutropenia and anemia.

In addition to having a high incidence of rheumatoid arthritis patients may have an autoimmune syndrome of hypergammaglobulinemia, circulating immune complexes and multiple autoantibodies.

The clinical presentation, immunologic, pathologic, molecular and genetic features of this disease with rheumatoid arthritis and Felty’s syndrome are indistinguishable.

No standard of care exists.

Responds to low dose oral methotrexate

Relative indolent disease as indicated by Osuji with a median survival of 14.5 years.

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