Recent Progress in the Understanding of Angioimmunoblastic T-cell Lymphoma

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  • Fujisawa Manabu
    Department of Hematology, Graduate School of Comprehensive Human Sciences, University of Tsukuba
  • Chiba Shigeru
    Department of Hematology, Graduate School of Comprehensive Human Sciences, University of Tsukuba Department of Hematology, Faculty of Medicine, University of Tsukuba Department of Hematology, University of Tsukuba Hospital
  • Sakata-Yanagimoto Mamiko
    Department of Hematology, Graduate School of Comprehensive Human Sciences, University of Tsukuba Department of Hematology, Faculty of Medicine, University of Tsukuba Department of Hematology, University of Tsukuba Hospital

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<p>Angioimmunoblastic T-cell lymphoma (AITL) has been classified as a subtype of mature T-cell neoplasms. The recent revision of the WHO classification proposed a new category of nodal T-cell lymphoma with follicular helper T (TFH)-cell phenotype, which was classified into three diseases: AITL, follicular T-cell lymphoma, and nodal peripheral T-cell lymphoma with TFH phenotype. These lymphomas are defined by the expression of TFH-related antigens, CD279/PD-1, CD10, BCL6, CXCL13, ICOS, SAP, and CXCR5. Although recurrent mutations in TET2, IDH2, DNMT3A, RHOA, and CD28, as well as gene fusions, such as ITK-SYK and CTLA4-CD28, were not diagnostic criteria, they may be considered as novel criteria in the near future. Notably, premalignant mutations, tumor-specific mutations, and mutations specific to tumor-infiltrating B cells were identified in AITL. Thus, multi-step and multi-lineage genetic events may lead to the development of AITL.</p>

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