Abstract
T-cell prolymphocytic leukemia (T-PLL) is a rare mature T-cell hematologic neoplasm with a very poor prognosis and limited treatment options to date. Single-agent alemtuzumab remains the first line of therapy for the treatment-naive and relapsed/refractory patients. Prospective clinical trials are difficult to conduct given that these patients have a short life expectancy after the initial diagnosis. As a result, researchers are implementing the use of targeted therapies in vitro and ex vivo followed by in vivo trials on a small subset of patients which are reviewed here. Newer approaches in the treatment of T-PLL are developing based on recognizing the cytogenetic phenotype of each patient and targeting the identified defective genes that are usually involved in the cell cycle regulation such as protooncogenes, tumor suppressors, and deoxyribonucleic acid (DNA) repair genes. These could potentially redirect the management in the near future and improve the overall survival (OS) and the progression-free survival (PFS) for these patients.
Highlights
BackgroundTcell prolymphocytic leukemia (T-PLL) is a very rare, mature T cell leukemia of small to medium size lymphocytes that shows post-thymic features
Close monitoring is recommended with complete blood count at monthly intervals along with the clinical examination [8]
Ex vivo response to venetoclax, significantly correlated with the expression of the B-cell lymphoma 2 (BCL2) protein
Summary
Tcell prolymphocytic leukemia (T-PLL) is a very rare, mature T cell leukemia of small to medium size lymphocytes that shows post-thymic features. Similar to the approach that combines a purine analog with alemtuzumab, a case was reported implementing the use of a purine analog (pentostatin) with venetoclax, a novel combination used in a patient with refractory and relapse disease, which resulted in a durable complete remission [27]. Targeted epigenetic therapies (such as EZH2 inhibitors), tumor suppressor (CHEK2, FBXW10, and SAMHD1) enhancers, P53 de-repressors, and many other rationale-based techniques are currently being studied with potential for future treatment in many hematologic malignancies including T-PLL [16, 33,34,35,36,37]
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