What Is T-Cell Prolymphocytic Leukemia?

T-cell prolymphocytic leukemia (T-PLL) is a rare and aggressive type of mature T-cell leukemia. It is characterized by the proliferation of small to medium-sized prolymphocytes (a type of white blood cell) with a mature T-cell phenotype (observable individual traits). T-PLL typically presents in older adults and is more common in men than in women. The disease is often aggressive, with rapid progression and resistance to chemotherapy. Patients with T-PLL often present with: generalized lymphadenopathy (swollen lymph nodes), hepatosplenomegaly (swelling of the liver or spleen), skin lesions, and marked by a high white blood cell count.

Why do people get T-Cell Prolymphocytic Leukemia?

The exact cause of T-cell prolymphocytic leukemia is not known. However, it is believed to be related to genetic mutations. The most common genetic abnormality in T-PLL is the inversion or translocation of chromosome 14, which leads to the overexpression of the TCL1 gene. This gene plays a role in the development and function of T-cells, and its overexpression may contribute to the development of T-PLL. T-cells are a type of white blood cell called lymphocytes. They help your immune system fight germs and protect you from disease.

Other factors that may contribute to the development of T-PLL include exposure to certain chemicals or radiation, a history of other blood disorders, and a weakened immune system. However, in many cases, the cause of T-PLL is not known. It is also important to note that T-PLL is not contagious and cannot be passed from person to person.

Who gets T-Cell Prolymphocytic Leukemia?

T-PLL is a rare T cell leukemia. The disease accounts for approximately 2% of cases of mature lymphocytic leukemia in adults. The pathology is common in elderly patients (older than 65 yrs) with an age range of 30 to 94 years. There is a slight male predominance with a male to female ratio of 1.33. Mutations of the ATM (ataxia-telangiectasia mutation) gene on 11q23 occur in 80% to 90% of the T-PLL population

Want to Learn More About T-Cell Prolymphocytic Leukemia?

Keep reading HealthTree for T-Cell Prolymphocytic Leukemia's 101 pages!

• How Is T-Cell Prolymphocytic Leukemia Diagnosed?
• How Long Will I Live With T-Cell Prolymphocytic Leukemia?
• What Are The Signs And Symptoms Of T-Cell Prolymphocytic Leukemia?
• How Is T-Cell Prolymphocytic Leukemia Staged And Classified?
• What Are Treatments For T-Cell Prolymphocytic Leukemia?