T-cell large granular lymphocyte leukemia pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [3]

T-cell large granular lymphocyte leukemia Microchapters
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Differentiating T-cell large granular lymphocyte leukemia from other Diseases
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Pathophysiology

The postulated cells of origin are a transformed CD8+ T-cell with clonal rearrangements of β chain T-cell receptor genes for the majority of cases and a CD8- T-cell with clonal rearrangements of γ chain T-cell receptor genes for a minority of cases.

Genetics

Clonal rearrangements of the T-cell receptor (TCR) genes are a necessary condition for the diagnosis of this disease. The gene for the β chain of the TCR is found to be rearranged more often than the γ chain of the TCR.

Gross Pathology

The leukemic cells of T-LGL can be found in peripheral blood, bone marrow, spleen, and liver. Nodal involvement is rare.

Microscopic Pathology

• The neoplastic lymphocytes seen in this disease are large in size with azurophilic granules that contains proteins involved in cell lysis such as perforin and granzyme B.
• Bone marrow involvement in this disease is often present, but to a variable extent. The lymphocytic infiltrate is usually interstitial, but a nodular pattern rarely occurs.