T-large granular lymphocytic leukemia

Sang Hyuk Park; Yoo Jin Lee; Youjin Kim; Hyun-Ki Kim; Ji-Hun Lim; Jae-Cheol Jo

doi:10.5045/br.2023.2023037

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Blood Res 2023; 58(S1):

Published online April 30, 2023

https://doi.org/10.5045/br.2023.2023037

T-large granular lymphocytic leukemia

Sang Hyuk Park^1#, Yoo Jin Lee^2#, Youjin Kim², Hyun-Ki Kim¹, Ji-Hun Lim¹, Jae-Cheol Jo²

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¹Department of Laboratory Medicine, ²Department of Hematology and Oncology, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Korea

Correspondence to : Jae-Cheol Jo, M.D., Ph.D.
Department of Hematology and Oncology, Ulsan University Hospital, University of Ulsan College of Medicine, 25 Daehakbyeongwon-ro, Dong-gu, Ulsan 44033, Korea
E-mail: jcjo@uuh.ulsan.kr; jcjo@ulsan.ac.kr

^#These authors contributed equally to this work.

Received: February 14, 2023; Revised: March 11, 2023; Accepted: March 14, 2023

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

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Abstract

T-cell large granular lymphocyte (T-LGL) leukemia is characterized by clonal expansion of cytotoxic T cells resulting in cytopenia. The proliferation of clonal LGLs is caused by prolonged antigenic stimulation, which leads to apoptotic dysregulation owing mainly to the constitutive activation of survival pathways, notably the JAK/STAT pathway. Understanding how leukemic T-LGL persists can aid in the development of future immunosuppressive therapies. In this review, we summarize the diagnosis and current standard of therapy for T-LGL leukemia, as well as recent advances in clinical trials.

Keywords T-cell large granular lymphocyte leukemia, Diagnosis, Treatment

Article

Review Article

Blood Res 2023; 58(S1): S52-S57

Published online April 30, 2023 https://doi.org/10.5045/br.2023.2023037

T-large granular lymphocytic leukemia

Sang Hyuk Park^1#, Yoo Jin Lee^2#, Youjin Kim², Hyun-Ki Kim¹, Ji-Hun Lim¹, Jae-Cheol Jo²

¹Department of Laboratory Medicine, ²Department of Hematology and Oncology, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Korea

Correspondence to:Jae-Cheol Jo, M.D., Ph.D.
Department of Hematology and Oncology, Ulsan University Hospital, University of Ulsan College of Medicine, 25 Daehakbyeongwon-ro, Dong-gu, Ulsan 44033, Korea
E-mail: jcjo@uuh.ulsan.kr; jcjo@ulsan.ac.kr

^#These authors contributed equally to this work.

Received: February 14, 2023; Revised: March 11, 2023; Accepted: March 14, 2023

Abstract

Keywords: T-cell large granular lymphocyte leukemia, Diagnosis, Treatment

Abstract

Fig 1.

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Figure 1.T-cell large granular lymphocyte (T-LGL) cells found in peripheral blood and bone marrow aspiration/ touch print. T-LGL cells found in peripheral blood (A and B, indicated with red arrows, ×1,000, Wright stain), bone marrow aspiration (C, indicated with red colored arrows, ×1,000, Wright stain), and touch print (D, indicated with red colored arrows, ×1,000, Wright stain) of a patient treated in the author’s hospital.

Blood Research 2023; 58: S52-S57https://doi.org/10.5045/br.2023.2023037

Fig 2.

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Figure 2.Schematic diagram describing the pathogenesis of T-cell large granular lymphocytes (T-LGL). Activation and expansion of the oligoclonal cytotoxic lymphocyte population are caused by stimulation with an unknown antigen. While STAT3 is activated and/or mutated, chronic antigen stimulation leads to the expansion of one dominant (monoclonal) cytotoxic lymphocyte population. Persistent clonal expansion of monoclonal cytotoxic lymphocytes occurs by dysregulation of Fas/FasL-mediated cell death, which results in resistance to apoptosis.

Blood Research 2023; 58: S52-S57https://doi.org/10.5045/br.2023.2023037