1. Academic Validation
  2. Molecular Pathogenesis of Peripheral T Cell Lymphoma

Molecular Pathogenesis of Peripheral T Cell Lymphoma

  • Curr Hematol Malig Rep. 2015 Dec;10(4):429-37. doi: 10.1007/s11899-015-0289-7.
Mamiko Sakata-Yanagimoto 1 Shigeru Chiba 2
Affiliations

Affiliations

  • 1 Department of Hematology, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan. [email protected].
  • 2 Department of Hematology, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan.
Abstract

Understanding the molecular pathogenesis of peripheral T cell lymphomas (PTCLs) has lagged behind that of B cell lymphomas due to disease rarity. However, novel approaches are gradually clarifying these mechanisms, and gene profiling has identified specific signaling pathways governing PTCL cell survival and growth. For example, genetic alterations have been discovered, including signal transducer and activator of transcription (STAT)3 and STAT5b mutations in several PTCLs, disease-specific Ras homolog family member A (RHOA) mutations in angioimmunoblastic T cell lymphoma (AITL), and recurrent translocations at the dual specificity Phosphatase 22 (DUSP22) locus in anaplastic lymphoma receptor tyrosine kinase (ALK)-negative anaplastic large cell lymphomas (ALCLs). Intriguingly, some PTCL-relevant mutations are seen in apparently normal blood cells as well as tumor cells, while Others are confined to tumor cells. These data have dramatically changed our understanding of PTCL origins: once considered to originate from mature T lymphocytes, some PTCLs are now believed to emerge from immature hematopoietic progenitor cells.

Keywords

B cell lymphomas; Peripheral T cell lymphomas (PTCLs); T cell lymphoma.

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