Open Access

REVIEW

T-cell prolymphocytic leukemia, a case report and review of the literature

LUIS MANUEL GONZáLEZ-RODRíGUEZ¹, LUIS MIGUEL JUáREZ-SALCEDO^1,*, JAVIER LOSCERTALES¹, EVA ARRANZ¹, JIMENA CANNATA-ORTIZ¹, JAVIER ORTIZ¹, MARIA JOSé LóPEZ DE LA OSA¹, ADRIáN ALEGRE¹, SAMIR DALIA^2,*

1 Hematology Department, La Princesa University Hospital, Madrid, 28006, Spain
2 Hematology/Oncology, Mercy Clinic Oncology and Hematology–Joplin, Misouri, MO 64804, USA

* Corresponding Authors: LUIS MIGUEL JUáREZ-SALCEDO. Email: ; SAMIR DALIA. Email:

Oncology Research 2025, 33(3), 505-517. https://doi.org/10.32604/or.2025.058175

Received 06 September 2024; Accepted 20 December 2024; Issue published 28 February 2025

Abstract

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 STAT5B 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.

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