T-prolymphocytic leukemia (T-PLL) is a rare and unusual hematological malignancy.
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Terminology
In the most recent version of the WHO classification, this is referred to as T-prolymphocytic leukemia rather than T-cell prolymphocytic leukemia.
Epidemiology
It represents around 2% of all mature lymphocytic leukaemias in adults over the age of 30 1. It usually affects older adults, with an average age at presentation being around 65 years. There may be a slight male predominance.
Clinical presentation
While there can be a potentially-wide clinical spectrum, many patients tend to present with hepatosplenomegaly and generalized lymphadenopathy.
Pathology
It is characterized by the proliferation of small- to medium-sized prolymphocytes of post-thymic origin. Many tissues can be involved, including peripheral blood, bone marrow, lymph nodes, liver, spleen, and skin.
Radiographic features
General
The imaging spectrum can also be variable with hepatosplenomegaly and generalized lymphadenopathy being common. Pleural effusions and cutaneous lesions may also be detectable in a small number of patients.
Treatment and prognosis
Clinical course is often very aggressive with poor response to conventional chemotherapy. Poor survival rates have usually been reported with the potential long-term curative treatment being hematopoietic stem cell transplantation. Treatment with purine analogs and alemtuzumab have been reported to have significantly higher response rates and improved survival 2,3.
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