KEGG   DISEASE: Adult T-cell leukemiaHelp
Entry
H00009                      Disease                                

Name
Adult T-cell leukemia
Description
Adult T-cell leukemia (ATL) is one of the most aggressive hematologic malignancies and is caused by human T-cell leukemia virus type I (HTLV-I). The HTLV-I Tax protein has been demonstrated to be the oncogenic protein of the virus. Tax may contribute to the process of carcinogenesis by a variety of mechanisms, including upregulating the expression of cellular genes involved in T cell growth and proliferation, including IL-2, IL-2R-alpha, and IL-15. However, ATL cells do not always need Tax expression in the later stage of leukemogenesis. Genetic and epigenetic changes should be implicated in such multistep leukemogenesis. Regarding genetic changes, mutation of p53, and deletion of p16 have been reported in ATL. Mutations of Fas gene are also reported in patients with ATL cells. However, such genetic changes were not frequently detected. In this regard, epigenetic change of p16/INK4A gene was more frequent in ATL cells, and accumulated according to the disease progression. This finding suggests that epigenetic change, including DNA methylation, plays an important role in the leukemogenesis of ATL.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of haematopoietic and lymphoid tissues
   H00009  Adult T-cell leukemia
Infectious diseases [BR:br08401]
 Viral infections
  Infections caused by retro-transcribing viruses
   H00009  Adult T-cell leukemia
Human diseases in ICD-10 classification [BR:br08403]
 2. Neoplasms (C00-D48)
  C81-C96  Malignant neoplasms, stated or presumed to be primary, of lymphoid, haematopoietic and related tissue
   C91  Lymphoid leukaemia
    H00009  Adult T-cell leukemia
Tumor markers [br08442.html]
 Commonly used tumor markers
  H00009
BRITE hierarchy
Pathway
HTLV-I infection
Viral carcinogenesis
Gene
p53 (mutation) [HSA:7157] [KO:K04451]
p16/INK4A (deletion, promoter methylation) [HSA:1029] [KO:K06621]
Fas (mutation) [HSA:355] [KO:K04390]
Pathogen
Human T-lymphotropic virus 1 [GN:T40003]
Marker
Lactate dehydrogenase [HSA:3939 3945 3948] [KO:K00016]
Drug
Cyclophosphamide [DG:DG00675]
Vincristine [DG:DG00690]
Doxorubicin [DG:DG00696]
Prednisone (CHOP) [DG:DG00094]
Rituximab [DR:D02994]
Comment
ICD-O: 9827/3, Tumor type: Adult T-cell leukaemia
Other DBs
ICD-10: 
MeSH: 
Reference
PMID:12910250 (description, gene)
  Authors
Matsuoka M
  Title
Human T-cell leukemia virus type I and adult T-cell leukemia.
  Journal
Oncogene 22:5131-40 (2003)
Reference
PMID:14686488 (gene)
  Authors
Yasunaga J, Matsuoka M
  Title
Leukemogenesis of adult T-cell leukemia.
  Journal
Int J Hematol 78:312-20 (2003)
Reference
PMID:10190897 (gene, tumor type)
  Authors
Maeda T, Yamada Y, Moriuchi R, Sugahara K, Tsuruda K, Joh T, Atogami S, Tsukasaki K, Tomonaga M, Kamihira S.
  Title
Fas gene mutation in the progression of adult T cell leukemia.
  Journal
J Exp Med 189:1063-71 (1999)
Reference
PMID:17387298 (carcinogen)
  Authors
Yasunaga J, Matsuoka M.
  Title
Human T-cell leukemia virus type I induces adult T-cell leukemia: from clinical aspects to molecular mechanisms.
  Journal
Cancer Control 14:133-40 (2007)
Reference
PMID:16362996 (carcinogen)
  Authors
Jarrett RF.
  Title
Viruses and lymphoma/leukaemia.
  Journal
J Pathol 208:176-86 (2006)
Reference
(drug)
  Authors
Papadakis MA and McPhee SJ |(ed)
  Title
Current Medical Diagnosis & Treatment 2015, Fifty-Fourth Edition
  Journal
McGraw Hill Education
Reference
  Authors
Chung CG, Poligone B
  Title
Cutaneous T cell Lymphoma: an Update on Pathogenesis and Systemic Therapy.
  Journal
Curr Hematol Malig Rep 10:468-76 (2015)

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