KEGG   DISEASE: Peripheral T cell lymphoma
Entry
H01892                      Disease                                

Name
Peripheral T cell lymphoma
  Subgroup
Anaplastic large-cell lymphoma [DS:H01601]
  Supergrp
Non-Hodgkin lymphoma [DS:H02418]
Description
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. With regard to the current WHO classification, leukemic, cutaneous, nodal, and extranodal PTCL are distinguished. The most common subtypes worldwide are the nodal T cell lymphomas. In the nodal T cell lymphomas, the major subtypes are PTCL, not otherwise specified (PTCL-NOS), angioimmunoblastic T cell lymphoma (AITL), anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL) and ALK-negative ALCL. Novel approaches are gradually clarifying the molecular pathogenesis of PTCLs. ALK gene translocation and dual specificity phosphatase 22 (DUSP22) gene translocation are seen in ALK-positive ALCL and ALK-negative ALCL, respectively. Recently, gene mutations in epigenetic regulators, including Ten-Eleven Translocation 2 (TET2), DNA methyltransferase 3A (DNMT3A), and isocitrate dehydrogenase 1 /2 (IDH1/2), are discovered in hematologic malignancies. The mutation frequencies were especially high in AITL and PTCL-NOS.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of haematopoietic and lymphoid tissues
   H01892  Peripheral T cell lymphoma
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Neoplasms of haematopoietic or lymphoid tissues
   Mature T-cell or NK-cell neoplasms
    2A90  Mature T-cell lymphoma, specified types, nodal or systemic
     H01892  Peripheral T cell lymphoma
Gene
NPM-ALK (translocation) [HSA:238] [KO:K05119]
DUSP22-IRF4 (translocation) [HSA:56940] [KO:K14165]
TET2 (mutation) [HSA:54790] [KO:K24309]
DNMT3A (mutation) [HSA:1788] [KO:K17398]
IDH1 (mutation) [HSA:3417] [KO:K00031]
IDH2 (mutation) [HSA:3418] [KO:K00031]
Drug
Pralatrexate [DR:D05589]
Brentuximab vedotin [DR:D09587] (CD30 expressing)
Romidepsin [DR:D06637]
Belinostat [DR:D08870]
Comment
9702/3 Mature T-cell lymphoma, NOS
9705/3 Angioimmunoblastic T-cell lymphoma
9714/3 Anaplastic large cell lymphoma, T cell and Null cell type
Other DBs
ICD-11: 2A90
ICD-10: C84.4 C84.6 C84.7 C86.5
MeSH: D016411
Reference
  Authors
Sandell RF, Boddicker RL, Feldman AL
  Title
Genetic Landscape and Classification of Peripheral T Cell Lymphomas.
  Journal
Curr Oncol Rep 19:28 (2017)
DOI:10.1007/s11912-017-0582-9
Reference
  Authors
Sakata-Yanagimoto M, Chiba S
  Title
Molecular Pathogenesis of Peripheral T Cell Lymphoma.
  Journal
Curr Hematol Malig Rep 10:429-37 (2015)
DOI:10.1007/s11899-015-0289-7
Reference
  Authors
Reimer P
  Title
New developments in the treatment of peripheral T-cell lymphoma - role of Belinostat.
  Journal
Cancer Manag Res 7:145-51 (2015)
DOI:10.2147/CMAR.S85351
Reference
  Authors
Sakata-Yanagimoto M
  Title
Multistep tumorigenesis in peripheral T cell lymphoma.
  Journal
Int J Hematol 102:523-7 (2015)
DOI:10.1007/s12185-015-1738-8
Reference
PMID:28622960 (drug)
  Authors
Feldman T, Farber CM, Choi K, Faria C, Goy A, Connors J, Paramanathan D, Kaur S, Schultz E, McGuire M, Goldberg SL
  Title
Treatment of Peripheral T-Cell Lymphoma in Community Settings.
  Journal
Clin Lymphoma Myeloma Leuk 17:354-361 (2017)
DOI:10.1016/j.clml.2017.05.001
Reference
PMID:27071634 (drug)
  Authors
Zhang Y, Xu W, Liu H, Li J
  Title
Therapeutic options in peripheral T cell lymphoma.
  Journal
J Hematol Oncol 9:37 (2016)
DOI:10.1186/s13045-016-0267-0
Reference
PMID:28115372 (drug)
  Authors
Broccoli A, Zinzani PL
  Title
Peripheral T-cell lymphoma, not otherwise specified.
  Journal
Blood 129:1103-1112 (2017)
DOI:10.1182/blood-2016-08-692566
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