REVIEW article

Front. Oncol., 19 May 2023

Sec. Cancer Metabolism

Volume 13 - 2023 | https://doi.org/10.3389/fonc.2023.1108729

Junction of the redox dynamic, orchestra of signaling, and altered metabolism in regulation of T- cell lymphoma

  • Biochemistry and Molecular Biology Laboratory, Centre for Advanced Study in Zoology, Institute of Science, Banaras Hindu University, Varanasi, India

Abstract

T-cell lymphoma is a hematologic neoplasm derived from the lymphoid lineage. It belongs to a diverse group of malignant disorders, mostly affecting the young population worldwide, that vary with respect to molecular features as well as genetic and clinical complexities. Cancer cells rewire the cellular metabolism, persuading it to meet new demands of growth and proliferation. Furthermore, the metabolic alterations and heterogeneity are aberrantly driven in cancer by a combination of genetic and non-genetic factors, including the tumor microenvironment. New insight into cancer metabolism highlights the importance of nutrient supply to tumor development and therapeutic responses. Importantly, oxidative stress due to an imbalance in the redox status of reactive species via exogenous and/or endogenous factors is closely related to multiple aspects of cancer. This alters the signaling pathways governed through the multiple intracellular signal transduction and transcription factors, leading to tumor progression. These oncogenic signaling molecules are regulated through different redox sensors, including nuclear factor-erythroid 2 related factor 2 (Nrf2), phase-II antioxidant enzyme, and NQO1 (NADPH quinone oxidoreductase (1). The existing understanding of the molecular mechanisms of T-cell lymphoma regulation through the cross-talk of redox sensors under the influence of metabolic vulnerability is not well explored. This review highlights the role of the redox dynamics, orchestra of signaling, and genetic regulation involved in T-cell lymphoma progression in addition to the challenges to their etiology, treatment, and clinical response in light of recent updates.

Introduction

Genetic mutations, which are fuel for cancer, have been linked to different human malignancies. Every day, hundreds of cells undergo mutation in the human body, but they do not all go through tumor formation. However, under adverse conditions, adaptive changes may cause cells to behave abnormally, changing survival, growth, and division patterns, which potentially disturbs cellular and social control (1). The multiple rewirings of the cell’s metabolic, genetic, and signaling pathways resulting in a very disruptive destiny for the cell’s social environment. Lymphoid cells, a critical component of the immune system, have been identified as emerging players associated with the progression of multiple forms of cancer, including lymphoma. Lymphoma is a malignant disease consisting of a large heterogeneous group of lymphocytic cells that reside within the lymph nodes, spleen, thymus, bone marrow, and other parts of the body. Broadly, it is classified into Hodgkin’s and non-Hodgkin’s lymphoma (2). Hodgkin’s lymphoma is named after Dr. Thomas Hodgkin who discovered this disease in 1832. Hodgkin’s lymphoma is classified on the basis of the presence of a specific type of cell called a ‘Reed-Sternberg cell’, which is absent in its parallel subtype non-Hodgkin’s lymphoma (2). According to the ‘5th edition of WHO classification of lymphocyte neoplasm 2022’, tumor heterogeneity represents around 115 different types of lymphoid descendants (3). Non-Hodgkin’s lymphoma is classified as abnormal clonal proliferation of T cells and B cells that lack Reed-Sternberg cells, where the majority of them (80–90%) arise from B-type lymphocytes and the rest (10–20%) originate from T lymphocyte or natural killer cells (4). Lymphocytes have a definite life span; however, under certain circumstances they start dividing, causing them to aggregate in lymph nodes, which results in swelling of the lymphatic system. Various immune-suppressed pathologies, including HIV, receiving high doses of chemotherapy, organ transplantation, stem cell therapy, and even infection with Epstein bar virus and Helicobactor pylori bacteria, may cause a predisposition to non-Hodgkin’s lymphoma (5). This review focused on types and subtypes of lymphoma, specifically T-cell Lymphoma, and their dynamic regulation under the influence of redox status, metabolic tuning, and signaling pathways.

T-cell lymphoma

The human body responds to foreign invaders with the help of lymphoid cells and the lymphoid system, including all cells of adaptive and innate immunity. T-cell lymphoma is hematologic neoplasm derived from the lymphoid lineage, which normally governs immune responses. Abnormality in this immune regulation has been reported in post-thymic or activated T lymphocytes. As per the report of the American Cancer Society, T-cell lymphoma accounts for about 10% of all lymphoma. WHO classified T-cell lymphoma into 39 types and 11 different categories (3). Different types of T-cell Lymphoma are listed in Table 1. Based on the involvement of bone marrow, it can be recognized as either lymphoma and/or acute lymphoblastic leukemia. It starts at the thymus site of T-cell maturation and develops into a tumor at the mediastinum and can spread to almost any part of the body, including the liver, bone marrow, gastrointestinal tract, spleen, skin, and brain (6).

Table 1

SNT-cell lymphomaSubtypes
1

Tumor-like lesions with T-cell predominance

Kikuchi-Fujimoto disease
Indolent T-lymphoblastic proliferation
Autoimmune lympho-proliferative syndrome
2
Precursor T-cell neoplasm
T-lymphoblastic leukemia/lymphoma
Early T-precursor lymphoblastic leukemia/lymphoma
3Mature T-cell and NK-cell neoplasm





a




Mature T-cell and NK-cell leukemiaT-prolymphocytic leukemia
Tlarge granular lymphocytic leukemia
NK-large granular lymphocytic leukemia
Adult T-cell leukemia/lymphoma
Sezary syndrome
Aggressive NK-cell leukemia








b







Primary cutaneous T-cell lymphomaPrimary cutaneous CD4-positive small or medium T-cell lympho-proliferative disorder
Primary cutaneous acral CD8-positive lympho-proliferative disorder
Mycosis fungoides
Primary cutaneous CD30-positive T-cell lympho-proliferative disorder: Lymphomatoid papulosis
Primary cutaneous CD30-positive T-cell lympho-proliferative disorder: Primary cutaneous anaplastic large cell lymphoma
Subcutaneous panniculitis-like T-cell lymphoma
Primary cutaneous gamma/delta T-cell lymphoma
Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma
Primary cutaneous peripheral T-cell lymphoma




c



Intestinal T-cell and NK-cell lymphoid proliferations and lymphomaIndolent T-cell lymphoma of the gastrointestinal tract
Indolent NK-cell lymphoproliferative disorder of the gastrointestinal tract
Enteropathy associated T-cell lymphoma
Monomorphic epitheliotropic intestinal T-cell lymphoma
Intestinal T-cell lymphoma
dHepatosplenic T-cell lymphomaHepatosplenic T-cell lymphoma



e


Anaplastic large cell lymphomaALK-positive anaplastic large cell lymphoma
ALK-negative anaplastic large cell lymphoma
Breast implant-associated anaplastic large cell lymphoma
Nodal T-follicular helper (TFH) cell lymphoma
fPeripheral T-cell lymphomaPeripheral T-cell lymphoma
gEBV-positive NK/T-cell lymphomaEBV-positive nodal T- and NK-cell lymphoma




h



EBV positive T-cell and NK-cell lymphomaSevere mosquito bite allergy
Extranodal NK/T-cell lymphoma
Hydroavacciniforme lympho-proliferative disorder
Systemic chronic active EBV disease
Systemic EBV-positive T-cell lymphoma of childhood

T-cell Lymphoma types.

“a-h” represents subdivision of mature T-cell and NK-cell leukemia.

Epithelial–mesenchymal transition (EMT) is initiated in a malignant T-cell lymphoma, which allows epithelial cells to be converted into mesenchymal cells and moves them into distinct locations through a process known as metastasis. Chemotherapy is the most preferred treatment option, being preferable over radiation and surgery. However, a plethora of side effects, including chemotherapy-induced peripheral neuropathy (CIPN) and immunosuppression, can be seen in many parts of the body, including the brain, where it may display lifelong persistence (79). The antioxidant defense system is crucial in maintaining cellular homeostasis. An imbalanced redox status due to high metabolic demand and the microenvironment of T-cells could be targeted for treatment.

Redox status and antioxidant defense system

Healthy cells routinely encounter immense amount of stress due to environmental (xenobiotic exposure, microbial exposure, etc.) and cellular (metabolic, immune function, etc.) responses in form of reactive oxygen species (ROS). These ROS are collectively defined under two subtypes, free radical species, including superoxide anions (O2•−), hydroxyl radicals (HO•), peroxyl (RO2•), and alkoxyl (RO•), and non-radical species, such as hydrogen peroxide (H2O2) (10). Accumulation of ROS cumulatively builds up in the cell in the form of oxidative stress. Oxidative stresses are imbalances between oxidants and antioxidants, leading to modification of biochemical properties of biomolecules in cells, which is balanced by an intracellular antioxidant defense system (11). Modulation of redox states contributed to multistage carcinogenesis either by a direct mechanism involving damage of DNA or indirectly by modulating cellular signal transduction (12, 13). ROS affect the function of multiple cellular proteins either by stabilizing them or modifying them in such a way that they can easily interact with their activator for their downstream target where they cause prolonged or constitutive activation of growth factors, cytokines, etc. NF-κB is a potent target of ROS that activates multiple transcription factors associated with cell cycle progression and survival (12). Elevated oxidative stress has been reported in many types of cancer cells where the redox changes have significant consequences. The application of antioxidants on T-cell lymphoma for therapeutic response has been extensively studied.

Models for T-cell lymphoma study

For future drug development and etiological investigation there is a need for a suitable model that can mimic maximum possible properties of problems from the preclinical to clinical grade. A wide range of preclinical models are being studied in research ranging from primary cultures, cell lines, xenografting, and patient derived xenografting (PDX) to more advanced organoid cultures including the patient-derived organoid (PDO). Due to the diversity and/or heterogeneity found in T-cell malignancy a wide variety of in vivo as well as in vitro models have been established (13, 14). Various animal models used in T-cell lymphoma are listed in Table 2. Our elucidation of T cells in this review is mainly dominated by a transplantable T-cell lymphoma model also known as Dalton’s lymphoma. It has shown maximum association with a range of clinical markers of T-cell lymphoma. A wide range of markers, including regulation of CD3+, massive depletion of immature CD4+, CD8+ and mature CD4+, CD8-, and CD8- along with impaired regulation of immunoregulatory cytokines such as IFN-ϒ, IL-10 and IL-2 for T-cell lymphoma, are listed in Table 3.

Table 2

SNTypeModelMechanismEffectReference
1Angioimmunoblastic T-cell lymphomaRoquin mouse modelA missense (M199R) San Roque mutation in the roquin geneIncrease of THF cell(15)
Tet2 gene trap mice modelA gene-trap vector inserted into the Tet2 section intronTHF- like phenotype(16)
G17V RHOA mouse modelBy stabilizing G17V RHOA gene expression either retroviral transduction, knockoutIncreases THF cell population(1719)
PDX Models of angioimmunoblastic T-cell lymphomaInoculation of cells from lymph node of AITL to NOG miceAITL-like disease(20)
2Anaplastic large T-cell lymphomaNPM1-ALK transgenic modelsBy transplanting bone marrow cells transduced with a retroviral vector carrying NPM1-ALK cDNA into lethally irradiated mice and using transgenic approachesResult in development of plasmacytoma, histiocytic malignancy, and large cell lymphoma from B-lineage(2126)
PDX Models of anaplastic large-cell lymphomasInoculation of CD30+ALCL cell from patient to SCID miceALCL, ALK+ disease(27)
3Human
T-cell lymphotropic virus type 1 adult T-cell leukemia/lymphoma
Mice expressing HTLV-1 viral proteinsUse of transgenics mice expressing Tax under the control of viral promoters HTLV1Development of mesenchymal tumor in nose, ear, foot, and tail(28)
PDX Models of adult T-cell leukemia/lymphomaXenograft of patient derived cell to SCID and NOD/SCID micelymphocytic infiltration in spleen, liver, lung, and other organs(29)
4Cutaneous T-cell lymphomaIL‐15 transgenic modelTransgenic mice model having increased IL-15 expression in CD4+T-cellFatal leukemia in skin(30, 31)
JAK3A572V mutant modelRetroviral induction of JAK3A572V mutant cDNA into 5-flurouracil-treated murine bone marrow cellsCD8+ leukemic condition in the skin(32, 33)
5Enteropathy-associated T-cell lymphomaSetd2cKOmicewere generated with Lck-Cre transgenic miceThrough transgenic and knockout processesIncreased no of ϒδ+T-cell in intraepithelial region(34)

T-cell lymphoma model.

Table 3

SNT-cell neoplasmAssociated markerReferences
1T-prolymphocytic leukemiaPositive for CD3 and CD7 but negative for CD5 and CD30(3537)
2T-large granular lympho-proliferativePositive for CD3, CD7, and CD8 but negative for CD4, CD5, and CD30(3840)
3Cutaneous ALCL (anaplastic large cell lymphoma)Positive for CD3 and CD30 but negative for CD8(4042)
4Hepatosplenic T-cell lymphomaPositive for CD3 and CD7 but negative for CD4, CD5, CD8, and CD30(38, 43, 44)
5Angioimmunoblastic T-cell lymphomaPositive for CD3 and CD5 but negative for CD7 and CD30(38, 45)
6Enteropathy associated T-cell lymphomaPositive for CD3, CD5, and CD7(46, 47)
7Adult T-cell leukemia/lymphomaPositive for CD3 and CD5 but not for CD7(48, 49)

T-cell lymphomas and their associated markers.

Regulation of T-cell lymphoma by ROS

ROS governs multiple cellular responses and are collectively referred to as “redox messengers” (10). These messengers further initiate a cascade of events in a small cytoplasmic pool to regulate different cellular functional proteins. Here, we describe some of such signaling events that contribute to progression of T-cell lymphoma in response to these messengers while highlighting signaling molecules involved in it.

Nrf2-NQO1 signaling

Nuclear factor erythroid 2-related factor 2 (Nrf2) is a leucine zipper transcription factor with 7-Neh (Nrf2 ECH homology) domains, each with a different function. Nrf2 is found in every cell type with basal level of activation and its level is regulated by 26s proteasomal degradation mediated by E3 ubiquitin ligase (Keap1-Cul3-Rbx) (50). There are around 200 genes with ARE (antioxidant response element) sequences which are regulated by Nrf2 (51, 52).

Cells are armored with intricate defense system to counter oxidative stress through the Keap1-Nrf2-ARE pathway (53). The Nrf2 protein is reported to be upregulated during oxidative stress, and knockout of Nrf2 results in severe deficiency in the coordination of the gene regulatory program with increased susceptibility to oxidative damage (54). Nrf2 is reported to be a double-edged sword with a dual role in controlling cancer (55). The role of Nrf2 in cancer is a little paradoxical, however, in T-cell lymphoma it is delimited to its traditional pathway where any deterioration in this pathway leads to carcinogenesis and restabilizing it results in the enhancement of lymphoma. Multiple drivers have been reported to activate or stabilize the expression of Nrf2. PKC (Protein kinase C) is one such sensor that stabilizes the translocation of Nrf2 from the cytosol to nucleus by phosphorylating it at the ser-40 position and facilitating its release from its cytosolic anchor Keap1 (56). Nrf2 protects the cell from oxidative damage by preventing cellular damage and halting cell cycle progression. Overexpression of PKC promotes tumor progression (57). Other accessory proteins such as ERK2 and GSK3β have also been reported to regulate Nrf2 activity (5866).

Genomic response by antioxidant signaling inducers suggest NAD(P)H:quinone oxidoreductase 1 (NQO1) and glutathione S-transferase (GST) are the two main target genes activated through Nrf2 in T-cell lymphoma (67, 68). NQO1 is a phase two antioxidant enzymes; it catalyzes the reduction of several environmental electrophonic contaminants and endogenous compounds, including quinones and nitro compounds (69). NQO1 under the regulation of Nrf2 plays a dual role in protection against carcinogenesis. Nrf2-dependent induction of NQO1 downregulated lipopolysaccharide (LPS)-induced expression of inflammatory cytokines, thereby impairing the inflammatory responses (70). It has also reported to induce apoptosis through p53. p53 is a tumor suppressor protein transcribed in response to cellular alteration like DNA damage and accumulates inside the cell to limit cell proliferation by initiating cell cycle arrest and apoptosis (71). Glutathione s-transferase (GST) is the second important target of the antioxidant-induced downstream response of Nrf2, which regulates metabolic detoxification of various electrophilic xenobiotic by forming a complex with them. Several isoforms of GST are reported to be expressed in a tissue-specific manner (72). The GST null phenotype is associated with an increased rate of cancer development (73). Nrf2 activation and its role in tumor suppression is depicted in Figure 1. Curcumin, an antioxidant, has shown anti-carcinogenic properties by elevating GSTα, GSTµ, and GSTπ activity (74).

Figure 1

NF-κB signaling

NF-κB represents a group of proteins that are very sensitive to any changes in the cellular redox profile and has a key role in cancer progression (75, 76). Normally, it remains sequestered in the cytoplasm along with inhibitory subunit Ikβ. ROS have been reported as both activators and inhibitory stimuli for NF-κB depending on the type of modification and site of action (7780). NF-kB regulates multiple proteins, including COX2, VEGE-A, TNF-α, and IL-6. The COX2 protein has been described as one of the major players linking inflammation to cancer through different signaling pathways (81, 82). Vascular endothelial growth factor-A (VEGF-A), another positive downstream of NF-κB, promotes angiogenesis by activating its receptor VEGF-R1 in endothelial cells of micro vessels in lymphoma (83). A schematic representation of the regulation of T-cell lymphoma by NF-κB is depicted in Figure 2. NF-κB has also been found to regulate inflammatory cytokines in a positive feedback mechanism such as TNF-α and IL-6 in T-cell malignant transformation (84).

Figure 2

PI3K-AKT signaling

PI3K signaling acts as master regulator in controlling cell survival, proliferation, and growth. It catalyzes the conversion of phosphatidyl inositol (4, 5)-bisphosphate (PIP2) to phosphatidylinositol (35) - triphosphate (PIP3), and this PIP3 initiates the localization of AKT to membrane and its subsequent activation by PDK1.ROS accumulation leads to phosphorylation of PI3K on tyrosine residues of a regulatory subunit (p85α) and thereby results in disassembly of it from its catalytic counterpart p110α (8587). The p110α triggers rapid transformation of PIP2 to PIP3. AKT activation resulting in cell transformation and generation of cancer phenotypes (88). AKT promotes cell survival by inhibiting proapoptotic protein BAD by phosphorylating it and preventing its interaction with BCL-xl (85, 8991). Regulation of T-cell lymphoma by the PI3k-AKT signaling pathway is depicted in Figure 3.

Figure 3

Conclusion and future perspective

From increasing experimental evidence, the role of ROS in tumorigenesis and its progression by regulating and/or altering the cellular environment has become apparent, stabilizing it as a potent hallmark of cancer. Although we have discussed some of the pathway, their complete regulation machinery is unknown and needs more scientific exploration. Changes in cellular redox are stabilized to contribute toward cancer pathogenesis but specific redox levels and their origin are still matters of research. Associated signaling pathways could reveal the hurdle of tumor progression by providing a target for drug development and help us better understand the etiology of cancer progression.

Statements

Author contributions

S.S.: writing-original draft and writing- review and editing. A.K.M.: conception and design, writing-original draft, writing-review and editing, administrative, technical, and material support. All authors listed have made a substantial, direct, and intellectual contribution to the work, and approved it for publication.

Acknowledgments

Research was supported by Institution of Eminence (IOE), Banaras Hindu University, Varanasi, India. SS thanks UGC, India for their Junior Research Fellowship.

Conflict of interest

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Publisher’s note

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Summary

Keywords

T-cell lymphoma, redox status, cancer metabolism, Nrf2, NF-κB, signaling pathways

Citation

Singh S and Maurya AK (2023) Junction of the redox dynamic, orchestra of signaling, and altered metabolism in regulation of T- cell lymphoma. Front. Oncol. 13:1108729. doi: 10.3389/fonc.2023.1108729

Received

26 November 2022

Accepted

21 March 2023

Published

19 May 2023

Volume

13 - 2023

Edited by

Santosh Kumar, National Institute of Technology Rourkela, India

Reviewed by

Yiqing Li, Department of Hematology, Sun Yat-sen University, China; Raj Kumar Koiri, Dr. Harisingh Gour Central University, India

Updates

Copyright

*Correspondence: Akhilendra Kumar Maurya,

This article was submitted to Cancer Metabolism, a section of the journal Frontiers in Oncology

Disclaimer

All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.

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