KEGG   DISEASE: B-cell acute lymphoblastic leukemia
H00001                      Disease                                
B-cell acute lymphoblastic leukemia;
B-cell acute lymphocytic leukemia
Philadelphia chromosome positive acute lymphoblastic leukemia
B-cell lymphoblastic lymphoma
Non-Hodgkin lymphoma [DS:H02418]
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). In the case of B-ALL, numerous reports have demonstrated that recurring genetic abnormalities are associated with sufficiently unique clinical, immunophenotypic, and/or prognostic features so that they can be considered as distinct entities. The most common rearrangements observed in B-ALL are the t(12;21) (p13;q22) rearrangement resulting in expression of the ETV6-RUNX1 fusion (TEL-AML1); the t(1;19) (q23;p13) translocation that results in expression of the TCF3 (E2A) fusion partner, (also known as TCF3) TFPT-PBX1 fusion (E2A-PBX); the t(9;22) (q34;q11.2) "Philadelphia" chromosome resulting in expression of the BCR-ABL1 fusion; and rearrangements of MLL (also known as KMT2A) at 11q23 to a diverse array of fusion partners. If none of these specific genetic abnormalities are found, the designation of "B lymphoblastic leukemia/lymphoma, not otherwise specified," is appropriate.
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Neoplasms of haematopoietic or lymphoid tissues
   Precursor lymphoid neoplasms
    2A70  Precursor B-lymphoblastic neoplasms
     H00001  B-cell acute lymphoblastic leukemia
Tumor markers [br08442.html]
BCR-ABL (translocation) [HSA:25] [KO:K06619]
MLL-AF4 (translocation) [HSA:4297 4299] [KO:K09186 K15184]
E2A-PBX1 (translocation) [HSA:6929 5087] [KO:K09063 K09355]
TEL-AML1 (translocation) [HSA:861] [KO:K08367]
c-MYC (rearrangement) [HSA:4609] [KO:K04377]
CRLF2 (rearrangement) [HSA:64109] [KO:K05078]
PAX5 (rearrangement) [HSA:5079] [KO:K09383]
Cyclophosphamide [DR:D00287]
Methotrexate [DR:D00142]
Mercaptopurine [DR:D00161]
Clofarabine [DR:D03546]
Vincristine sulfate [DR:D02197] (Philadelphia chromosome-negative)
Teniposide [DR:D02698]
Doxorubicin hydrochloride [DR:D01275]
Daunorubicin hydrochloride [DR:D01264]
Mitoxantrone hydrochloride [DR:D02166]
Imatinib mesylate [DR:D01441] (Philadelphia chromosome positive)
Dasatinib [DR:D06414] (Philadelphia chromosome positive)
Ponatinib hydrochloride [DR:D09951] (T315I positive, Philadelphia chromosome positive)
Pemigatinib [DR:D11417] (FGFR1 rearrangement)
Inotuzumab ozogamicin [DR:D08933]
Blinatumomab [DR:D09325] (Philadelphia chromosome-negative)
Tisagenlecleucel-T [DR:D11386]
Asparaginase [DR:D02997]
Pegaspargase [DR:D05387]
Enocitabine [DR:D01633]
Calaspargase pegol [DR:D10096]
Other DBs
ICD-11: 2A70
ICD-10: C83.5 C91.0
MeSH: D054198
Roberts KG, Mullighan CG
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Nat Rev Clin Oncol 12:344-57 (2015)
Mullighan CG
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Cornell RF, Palmer J
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Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, Bloomfield CD, Cazzola M, Vardiman JW
The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia.
Blood 127:2391-405 (2016)
Harrison JD, Muirhead CR.
Quantitative comparisons of cancer induction in humans by internally deposited radionuclides and external radiation.
Int J Radiat Biol 79:1-13 (2003)

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