Research Topic

Mature T Cell Neoplasms: The Unmet Need for Understanding their Pathophysiology and Optimal Treatment

About this Research Topic

Peripheral T cell lymphomas (PTCLs) are a group of rare lymphomas originating from mature (i.e., post-thymic or “peripheral”) T lymphocytes and NK cells, which comprise 10–15% of all non-Hodgkin lymphomas (NHL). With the exception of a few relatively indolent entities, they are generally aggressive with poor clinical outcomes. Currently there is no widely accepted standard of care for these difficult and rare disease entities compared to their B cell counterpart, not only due to rare incidence but also due to our lack of understanding of their pathophysiology.

Further research into the identification and development of active and biologically rational therapies to cure PTCL at high rates is required to improve patient outcome. Therapeutic targeting of biological pathways implicated in the pathogenesis of PTCL, such as T-cell receptor signaling, epigenetic deregulation, and pathways such as JAK/STAT, phosphatidylinositol 3 (Pi3) kinase, or mammalian target of rapamycin (mTOR) activation, has been postulated as a promising approach.

JAK/STAT signaling is aberrantly activated in many T cell neoplasms via multiple mechanisms, including inappropriate autocrine and paracrine cytokine stimulation as well as activating mutations. Abnormal activation of this system was pervasive in diverse T cell malignancies assessed by pSTAT3/pSTAT5 phosphorylation status in isolated cells. Thus, the JAK/STAT pathway appears to play a major role in the development of many T cell neoplasms which suggests potential novel therapeutic approaches.

Another peculiar feature of mature T cell neoplasms is their marked heterogeneity at the clinical, pathological, and molecular levels with many intriguing and distinctive subtypes. Recent advances in the molecular and genetic characterization of T-cell lymphomas have helped to delineate differences and similarities between the various subtypes. This emerging understanding of mutations, targets, and new drugs provides increased rationale for selecting agents for novel treatment of T cell lymphomas based on their mechanisms of action. Improved understanding of these differences will enable us to develop and devise subtype-specific and tailored treatment strategies involving different target agents.

In this Research Topic, we aim to collect a series of articles discussing advancing research being performed on mature T cell neoplasms at the pre-clinical, translational and clinical levels. This can be in the form of original articles, systematic reviews, case reports and review articles. We welcome the submission of Original Research, Case Reports and Review articles that cover, but are not limited to, the following topics:

1. Pathophysiology of T cell neoplasms: The role of JAK/STAT, PI3K, mTOR and other targetable pathways in these disease entities.
2. Conventional therapeutic approaches and the role of stem cell transplantation for PTCL treatment.
3. Novel therapeutic approaches including target agents for PTCL.
4. Challenging aspects of immunotherapy for PTCL: where do we stand?
5. CTCL: unique feature of T cell neoplasm, selection of treatment : skin vs systemic therapy and role of allogeneic stem cell transplantation
6. Rare types of PTCL: current treatment and future perspectives.


Important Note: All contributions to this Research Topic must be within the scope of the section and journal to which they are submitted, as defined in their mission statements. Frontiers reserves the right to guide an out-of-scope manuscript to a more suitable section or journal at any stage of peer review.

Peripheral T cell lymphomas (PTCLs) are a group of rare lymphomas originating from mature (i.e., post-thymic or “peripheral”) T lymphocytes and NK cells, which comprise 10–15% of all non-Hodgkin lymphomas (NHL). With the exception of a few relatively indolent entities, they are generally aggressive with poor clinical outcomes. Currently there is no widely accepted standard of care for these difficult and rare disease entities compared to their B cell counterpart, not only due to rare incidence but also due to our lack of understanding of their pathophysiology.

Further research into the identification and development of active and biologically rational therapies to cure PTCL at high rates is required to improve patient outcome. Therapeutic targeting of biological pathways implicated in the pathogenesis of PTCL, such as T-cell receptor signaling, epigenetic deregulation, and pathways such as JAK/STAT, phosphatidylinositol 3 (Pi3) kinase, or mammalian target of rapamycin (mTOR) activation, has been postulated as a promising approach.

JAK/STAT signaling is aberrantly activated in many T cell neoplasms via multiple mechanisms, including inappropriate autocrine and paracrine cytokine stimulation as well as activating mutations. Abnormal activation of this system was pervasive in diverse T cell malignancies assessed by pSTAT3/pSTAT5 phosphorylation status in isolated cells. Thus, the JAK/STAT pathway appears to play a major role in the development of many T cell neoplasms which suggests potential novel therapeutic approaches.

Another peculiar feature of mature T cell neoplasms is their marked heterogeneity at the clinical, pathological, and molecular levels with many intriguing and distinctive subtypes. Recent advances in the molecular and genetic characterization of T-cell lymphomas have helped to delineate differences and similarities between the various subtypes. This emerging understanding of mutations, targets, and new drugs provides increased rationale for selecting agents for novel treatment of T cell lymphomas based on their mechanisms of action. Improved understanding of these differences will enable us to develop and devise subtype-specific and tailored treatment strategies involving different target agents.

In this Research Topic, we aim to collect a series of articles discussing advancing research being performed on mature T cell neoplasms at the pre-clinical, translational and clinical levels. This can be in the form of original articles, systematic reviews, case reports and review articles. We welcome the submission of Original Research, Case Reports and Review articles that cover, but are not limited to, the following topics:

1. Pathophysiology of T cell neoplasms: The role of JAK/STAT, PI3K, mTOR and other targetable pathways in these disease entities.
2. Conventional therapeutic approaches and the role of stem cell transplantation for PTCL treatment.
3. Novel therapeutic approaches including target agents for PTCL.
4. Challenging aspects of immunotherapy for PTCL: where do we stand?
5. CTCL: unique feature of T cell neoplasm, selection of treatment : skin vs systemic therapy and role of allogeneic stem cell transplantation
6. Rare types of PTCL: current treatment and future perspectives.


Important Note: All contributions to this Research Topic must be within the scope of the section and journal to which they are submitted, as defined in their mission statements. Frontiers reserves the right to guide an out-of-scope manuscript to a more suitable section or journal at any stage of peer review.

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Submission Deadlines

15 January 2018 Abstract
16 April 2018 Manuscript

Participating Journals

Manuscripts can be submitted to this Research Topic via the following journals:

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Topic Editors

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Submission Deadlines

15 January 2018 Abstract
16 April 2018 Manuscript

Participating Journals

Manuscripts can be submitted to this Research Topic via the following journals:

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