T-cell large granular lymphocyte leukemia pathophysiology
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [3]
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Overview
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.[1]
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.[2]
- 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.
References
- ↑ [1] Vie H, Chevalier S, Garand R, Moisan JP, Praloran V, Devilder MC, Moreau JF, Soulillou JP. "Clonal expansion of lymphocytes bearing the gamma delta T-cell receptor in a patient with large granular lymphocyte disorder." Blood. 1989 Jul;74(1):285-90. PMID: 2546620
- ↑ [2] Semenzato G, Zambello R, Starkebaum G, Oshimi K, Loughran TP Jr. "The lymphoproliferative disease of granular lymphocytes: updated criteria for diagnosis." Blood. 1997 Jan 1;89(1):256-60. PMID: 8978299