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T-prolymphocytic leukemia/T-cell lymphocytic leukemia- Kiel
An aggressive T-cell leukemia characterized by the proliferation of small to medium sized prolymphocytes with a mature post-thymic T-cell phenotype involving the peripheral blood, bone marrow, lymph nodes, liver, spleen and skin.
T-cell prolymphocytic leukemia (T-PLL) is an aggessive T-cell leukemia. Leukemic T-cells are found in the peripheral blood, bone marrow, lymph nodes, spleen, liver and sometimes skin. Cutaneous involvement consists of perivascular or more diffuse dermal infiltrations without epidermotropism. The spleen histology shows dense red pulp infiltration which invades the spleen capsule, blood vessels and atrophied white pulp. In lymph nodes, the involvement is diffuse and tends to predominate in the paracortical areas, sometimes with sparing of follicles. Prominent high-endothelial venules may be numerous and are often infiltrated by neoplastic cells.
Definitive Diagnostic Methods
Bone Marrow biopsy
Peirpheral blood smear
Inversion chromosome 14 with breakpoints in the long arm at q11 and q32