The TCL1 gene is involved in the generation and/or manifestation of mature forms of leukemias, mainly in the T-Prolymphocytic leukemia (T-PLL) and in chronic lymphocytic leukemia (B-CLL).
Clinical Characteristics of the T-PLL
T-PLL is a disease that represents 20 % of prolymphocytic leukemias. It occurs at an advanced age of 70–80 years, with a slight male predominance. It is, however, quite frequent in patients with the immunodeficiency syndrome ataxia-telangiectasia (AT) (1–5 % of these patients develop it). Clinically, it is accompanied by splenomegaly (75 %), hepatomegaly (42 %), lymphadenopathy (55 %), and a high blood count (>200 × 109/L), with a very bad survival rate (<8 months). It involves mature T-cells (CD4+ in the 65 % but also CD8+ and CD4+ CD8+); T-cell prolymphocytes usually express CD3, CD5, and CD7. Morphologically, the T-prolymphocyte is smaller than its B-counterpart, usually with a single, sometimes irregular nucleolus. Chromosomal...