Large Granular Lymphocyte Leukemia

Abstract

Learning ObjectivesAfter completing this course, the reader will be able to:1. Discuss the basic principles of molecular and cellular biology of LGL leukemia. Describe distinct clinical entities among disorders ofLGLs.3. Discuss the diagnostic criteria for T-cell LGL leukemia.

T Cell Large Granular Lymphocytic Leukemia in Association with Sjögren’s Syndrome

“…Such an association has been frequently reported with rheumatoid arthritis or with less specific autoimmune features such as positivity to autoantibodies (e.g. antinuclear antibodies, antineutrophils) in the absence of a clear autoimmune disease [2]. Here, we report a case of T cell LGL leukemia in which a concurrent diagnosis of Sjögren’s syndrome was made.…”

“…The most common autoimmune disorder associated with T cell LGL leukemia is rheumatoid arthritis, but other autoimmune disorders have been described in the literature [2]. These include cases of thrombotic thrombocytopenic purpura, pure red cell aplasia, Behcet’s syndrome and Sjögren’s syndrome [9,10].…”

T-cell large granular lymphocytic (T-LGL) leukemia: a single institution experience

“…Despite the fact that T-LGL leukemia is an indolent disease that requires no treatment at the time of presentation in the majority of patients, 70% will ultimately require therapy at a later date. The most common indications for therapy include life-threatening infections, severe neutropenia, symptomatic anemia or thrombocytopenia, and severe B symptoms [2,9]. Because of the rarity of T-LGL leukemia, very few prospective clinical trials have been reported, and current treatment strategies are based on anecdotal case reports and small retrospective studies.…”

“…Because of the rarity of T-LGL leukemia, very few prospective clinical trials have been reported, and current treatment strategies are based on anecdotal case reports and small retrospective studies. In previous reports, treatment with immunomodulation, corticosteroids, low-dose methotrexate, cyclophosphamide, and cyclosporine A with or without purine analogues are all described [2,[9][10][11][12][13][14][15][16][17][18][19][20][21][22].…”

“…The clinical course of T-LGL leukemia is generally indolent; major indications for commencement of treatment include severe neutropenia, recurrent infections, and symptomatic anemia and thrombocytopenia, which indicate aggressive T-LGL leukemia. Various therapeutic options have been described, but there are no generally accepted guidelines for treatment [1,2,9].…”