KEGG   DISEASE: T lymphoblastic leukemia/lymphomaHelp
Entry
H00002                      Disease                                

Name
T lymphoblastic leukemia/lymphoma
Description
Acute lymphocytic leukemia (ALL) is a clonal stem cell malignancy of excessive lymphoblast proliferation. It is now understood that ALL and lymphoblastic lymphoma are the same disease entities at the morphologic and immunophenotypic levels and classified as either B- and T-cell lymphoblastic leukemia/lymphoma (B-ALL and T-ALL). T-ALL comprises 15% of paediatric and 25% of adult ALL cases. T cell transformation is a multi-step process in which different genetic alterations cooperate to alter the normal mechanisms that control cell growth, proliferation, survival, and differentiation during thymocyte development. In this context, constitutive activation of NOTCH1 signaling is the most prominent oncogenic pathway in T cell transformation. In addition, T-ALLs characteristically show the translocation and aberrant expression of transcription factor oncogenes. These oncogenic transcription factors include T-cell leukaemia homeobox protein 1 (TLX1 also known as HOX11), TLX3 (HOX11L2), LYL1, TAL1 and MLL.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of haematopoietic and lymphoid tissues
   H00002  T lymphoblastic leukemia/lymphoma
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Neoplasms of haematopoietic or lymphoid tissues
   Precursor lymphoid neoplasms
    2A71  Precursor T-lymphoblastic neoplasms
     H00002  T lymphoblastic leukemia/lymphoma
Tumor markers [br08442.html]
 H00002
BRITE hierarchy
Pathway
hsa05202  Transcriptional misregulation in cancer
Network
N00120  MLL-ENL fusion to transcriptional activation
N00121  LMO2-rearrangement to transcriptional activation
N00122  LMO2-rearrangement to transcriptional repression
N00144  TLX1 rearrangement to transcriptional repression
Gene
NOTCH1 (mutation) [HSA:4851] [KO:K02599]
TAL1 (overexpression) [HSA:6886] [KO:K09068]
LYL1 (expression) [HSA:4066] [KO:K15604]
MLL-ENL (translocation) [HSA:4297] [KO:K09186]
HOX11 (translocation) [HSA:3195] [KO:K09340]
MYC (translocation) [HSA:4609] [KO:K04377]
LMO2 (translocation) [HSA:4005] [KO:K15612]
HOX11L2 (translocation) [HSA:30012] [KO:K15607]
PICALM-MLLT10 (translocation) [HSA:8028]
Carcinogen
*Thorium-232 and its decay products, administered intravenously as a colloidal dispersion of thorium232 dioxide
*: Although this is a risk factor for ALL in general, the strength of association with different histological tumor types is not well understood.
Drug
Cyclophosphamide [DR:D00287]
Carmustine [DR:D00254]
Methotrexate [DR:D00142]
Mercaptopurine [DR:D00161]
Clofarabine [DR:D03546]
Nelarabine [DR:D05134]
Vincristine sulfate [DR:D02197] (Philadelphia chromosome-negative)
Teniposide [DR:D02698]
Doxorubicin hydrochloride [DR:D01275]
Daunorubicin hydrochloride [DR:D01264]
Mitoxantrone hydrochloride [DR:D02166]
Asparaginase [DR:D02997]
Pegaspargase [DR:D05387]
Enocitabine [DR:D01633]
Comment
ICD-O-3.1: 9837/3 T lymphoblastic leukemia/lymphoma
Other DBs
ICD-11: 2A71
ICD-10: C83.5 C91.0
MeSH: D054218
Reference
  Authors
Roberts KG, Mullighan CG
  Title
Genomics in acute lymphoblastic leukaemia: insights and treatment implications.
  Journal
Nat Rev Clin Oncol 12:344-57 (2015)
DOI:10.1038/nrclinonc.2015.38
Reference
  Authors
Teitell MA, Pandolfi PP
  Title
Molecular genetics of acute lymphoblastic leukemia.
  Journal
Annu Rev Pathol 4:175-98 (2009)
DOI:10.1146/annurev.pathol.4.110807.092227
Reference
  Authors
Van Vlierberghe P, Ferrando A
  Title
The molecular basis of T cell acute lymphoblastic leukemia.
  Journal
J Clin Invest 122:3398-406 (2012)
DOI:10.1172/JCI61269
Reference
  Authors
Cornell RF, Palmer J
  Title
Adult acute leukemia.
  Journal
Dis Mon 58:219-38 (2012)
DOI:10.1016/j.disamonth.2012.01.011
Reference
  Authors
Kraszewska MD, Dawidowska M, Szczepanski T, Witt M
  Title
T-cell acute lymphoblastic leukaemia: recent molecular biology findings.
  Journal
Br J Haematol 156:303-15 (2012)
DOI:10.1111/j.1365-2141.2011.08957.x
Reference
PMID:27069254 (tumor type)
  Authors
Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, Bloomfield CD, Cazzola M, Vardiman JW
  Title
The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia.
  Journal
Blood 127:2391-405 (2016)
DOI:10.1182/blood-2016-03-643544
Reference
PMID:12556326 (carcinogen)
  Authors
Harrison JD, Muirhead CR.
  Title
Quantitative comparisons of cancer induction in humans by internally deposited radionuclides and external radiation.
  Journal
Int J Radiat Biol 79:1-13 (2003)
DOI:10.1080/0955300021000038671

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