KEGG   DISEASE: Adult T-cell leukemia
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 1 (HTLV-1). The HTLV-1 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 ssRNA-RT viruses
   H00009  Adult T-cell leukemia
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
     H00009  Adult T-cell leukemia
Tumor markers [br08442.html]
 H00009
Pathway
hsa05166  Human T-cell leukemia virus 1 infection
Network
nt06160  Human T-cell leukemia virus 1 (HTLV-1)
nt06507  TGFB signaling
nt06518  JAK-STAT signaling
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]
Other DBs
ICD-11: 2A90.5
ICD-10: C91.5
MeSH: D015459
Reference
  Authors
Matsuoka M
  Title
Human T-cell leukemia virus type I and adult T-cell leukemia.
  Journal
Oncogene 22:5131-40 (2003)
DOI:10.1038/sj.onc.1206551
Reference
  Authors
Yasunaga J, Matsuoka M
  Title
Leukemogenesis of adult T-cell leukemia.
  Journal
Int J Hematol 78:312-20 (2003)
DOI:10.1007/BF02983555
Reference
  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)
DOI:10.1084/jem.189.7.1063
Reference
  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)
DOI:10.1177/107327480701400206
Reference
  Authors
Jarrett RF.
  Title
Viruses and lymphoma/leukaemia.
  Journal
J Pathol 208:176-86 (2006)
DOI:10.1002/path.1905

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