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Clinical manifestations, pathologic features, and diagnosis of T cell large granular lymphocyte leukemia

Thierry Lamy, MD, PhD
Thomas P Loughran, Jr, MD
Section Editor
Richard A Larson, MD
Deputy Editor
Alan G Rosmarin, MD


Large granular lymphocyte (LGL) leukemia is a clonal disease of the large granular lymphocyte characterized by peripheral blood and marrow lymphocytic infiltration with LGLs, splenomegaly, and cytopenias, most commonly neutropenia.

The LGL is a morphologically distinct lymphoid subtype that is larger than most circulating lymphocytes, and has characteristic azurophilic granules containing acid hydrolases (picture 1). LGLs comprise 10 to 15 percent of normal peripheral blood mononuclear cells. The absolute number of LGLs in the peripheral blood of normal subjects is 200 to 400/microL.

LGLs arise from two major lineages [1,2]:

Approximately 85 percent are CD3 positive, CD57 positive, CD56 negative T cells, representing in vivo antigen-activated effector-memory cytotoxic T cells (see 'Pathogenesis' below).

The remaining 15 percent are CD3-, CD56+ natural killer (NK) cells.

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Literature review current through: Nov 2017. | This topic last updated: Sep 09, 2016.
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