KEGG   DISEASE: H00009Help
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
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-cell lymphotropic virus type I [RS:NC_001436]
Marker
Survivin [HSA:332]
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
PMID:15293568 (marker)
  Authors
Sugahara K, Uemura A, Harasawa H, Nagai H, Hirakata Y, Tomonaga M, Murata K, Sohda H, Nakagoe T, Shibasaki S, Yamada Y, Kamihira S.
  Title
Clinical relevance of survivin as a biomarker in neoplasms, especially in adult T-cell leukemias and acute leukemias.
  Journal
Int J Hematol 80:52-8 (2004)
Reference
PMID:12613509 (marker)
  Authors
Nakayama K, Kamihira S.
  Title
Survivin an important determinant for prognosis in adult T-cell leukemia: a novel biomarker in practical hemato-oncology.
  Journal
Leuk Lymphoma 43:2249-55 (2002)
Reference
(drug)
  Authors
Papadakis MA, McPhee SJ (ed).
  Title
Current Medical Diagnosis & Treatment 2015, Fifty-Fourth Edition
  Journal
The McGraw-Hill Companies, Inc. (2014)

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