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Molecular Insights Into Pathogenesis of Peripheral T Cell Lymphoma: a Review

Current Hematologic Malignancy Reports volume 13, pages 318–328 (2018)Cite this article

Abstract

Purpose of Review

Peripheral T cell lymphoma (PTCL) is a heterogeneous group of lymphoproliferative neoplasms, with at least 29 distinct entities described in current WHO classification. Using present diagnostic approaches, more than a third of PTCL cases cannot be classified, hence designated as PTCL-not otherwise specified (PTCL-NOS). Herein, we summarize the current genomic findings and their role in the molecular pathogenesis in different PTCL entities.

Recent Findings

Gene expression profiling (GEP) studies have identified distinct molecular signatures for accurate diagnosis and elucidated oncogenic pathways enriched in major PTCL entities. Furthermore, genomic characterization has identified recurrent somatic mutations and potential therapeutic targets. Further efforts are underway to develop genetically faithful murine models.

Summary

GEP studies have identified molecular subgroups of PTCL, characterized by distinct genetic and epigenetic alterations. Understanding the molecular mechanisms of T cell lymphomagenesis using in vivo model will help to reveal novel therapeutic targets.

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Author notes

  1. Waseem Lone and Aisha Alkhiniji contributed equally to this work.

Affiliations

  1. Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE, 68198-6842, USA

    Waseem Lone, Aisha Alkhiniji & Javeed Iqbal

  2. Department of Internal Medicine, Division of Oncology & Hematology, University of Nebraska Medical Center, Omaha, NE, 68198-6842, USA

    Jayadev Manikkam Umakanthan

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  1. Waseem Lone
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  2. Aisha Alkhiniji
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  3. Jayadev Manikkam Umakanthan
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Correspondence to Javeed Iqbal.

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Waseem Lone, Aisha Alkhiniji, Jayadev Manikkam Umakanthan, and Javeed Iqbal declare they have no conflict of interests.

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This article is part of the Topical Collection on T Cell and Other Lymphoproliferative Malignancies

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Lone, W., Alkhiniji, A., Manikkam Umakanthan, J. et al. Molecular Insights Into Pathogenesis of Peripheral T Cell Lymphoma: a Review. Curr Hematol Malig Rep 13, 318–328 (2018). https://doi.org/10.1007/s11899-018-0460-z

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Keywords

  • PTCL
  • Peripheral T cell lymphoma-not otherwise specified
  • Angioimmunoblastic T cell lymphoma
  • Anaplastic large cell lymphoma
  • Gene expression profiling
  • Molecular signature