T-Cell Prolymphocytic Leukemia

Excerpt

T-PLL (T Prolymphocytic leukemia) is a rare, aggressive T-cell leukemia characterized by the proliferation of small to medium-sized prolymphocytes that show a mature T-cell phenotype. The average age of patients with T-PLL is 65 (the age range is 30 to 94 years). Generally, it comprises 2% of mature lymphocyte leukemias. It has a rapid doubling time and a doleful course with a median survival of 1 year. Between 20 and 30% of patients initially present with inactive disease, but they always progress to an active format within 2 years. T-PLL is often widespread at diagnosis and involves the peripheral blood, bone marrow, lymph nodes, liver, spleen, and skin. The name "prolymphocyte" is inaccurate, as the tumor cells in this disease are of post-thymic T cell origin.

After confirming the diagnosis, the next clinical objective is determining the disease's activity as the only active disease is treated. Chemotherapy's efficacy is poor. Agents that can offer a response cannot provide long-term control. Bone marrow transplantation can provide a long-term response but typically only in patients with a good complete response (CR) to induction chemotherapy, those in good condition/health, and young enough to allow a bone marrow transplantation protocol.