TY  - EJOU
AU  - GONZáLEZ-RODRíGUEZ, LUIS MANUEL 
AU  - JUáREZ-SALCEDO, LUIS MIGUEL 
AU  - LOSCERTALES, JAVIER 
AU  - ARRANZ, EVA 
AU  - CANNATA-ORTIZ, JIMENA 
AU  - ORTIZ, JAVIER 
AU  - OSA, MARIA JOSé LóPEZ DE LA 
AU  - ALEGRE, ADRIáN 
AU  - DALIA, SAMIR 

TI  - T-cell prolymphocytic leukemia, a case report and review of the literature
T2  - Oncology Research

PY  - 2025
VL  - 33
IS  - 3
SN  - 1555-3906

AB  - T-prolymphocytic leukemia is a rare and aggressive hematological malignancy characterized by the clonal proliferation of mature lymphoid T-cells. The pathogenesis of T-PLL is closely linked to specific chromosomal abnormalities, primarily involving the proto-oncogene T-cell leukemia/lymphoma 1 gene family. Recent advancements in molecular profiling have identified additional genomic aberrations, including those affecting the Janus kinase/signal transducers and activators of transcription (JAK/STAT) signaling pathway. This case report presents a patient with T-prolymphocytic leukemia whose cytogenetic and molecular analysis revealed a t(X;14)(q28;q11.2) translocation and a <i>STAT5B</i> mutation. Here, we aim to review the genetic and molecular underpinnings of T-prolymphocytic leukemia, as well as current treatment options, with a focus on the anti-CD52 monoclonal antibody alemtuzumab and JAK inhibitors. While alemtuzumab followed by allogeneic hematopoietic stem cell transplantation remains the standard of care for eligible patients, its efficacy is limited and many patients are ineligible. Emerging therapeutic approaches, such as JAK/STAT inhibitors, offer promising potential for improving patient outcomes.
KW  - T-prolymphocytic leukemia (T-PLL); MTCP1; JAK/STAT; STAT5B; Alemtuzumab

DO  - 10.32604/or.2025.058175