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Infant Acute Lymphoblastic Leukemia with KMT2A Rearrangement

MedGen UID:
890208
•Concept ID:
C4054729
•
Neoplastic Process
Synonym: Infant Acute Lymphoblastic Leukemia with MLL Rearrangement

Definition

An acute lymphoblastic leukemia with rearrangement of the KMT2A gene that occurs in infancy. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVInfant Acute Lymphoblastic Leukemia with KMT2A Rearrangement

Professional guidelines

PubMed

Genetic alterations and MRD refine risk assessment for KMT2A-rearranged B-cell precursor ALL in adults: a GRAALL study.
Kim R, Bergugnat H, Pastoret C, Pasquier F, Raffoux E, Larcher L, Passet M, Grardel N, Delabesse E, Kubetzko S, Caye-Eude A, Meyer C, Marschalek R, Lafage-Pochitaloff M, Thiebaut-Bertrand A, Balsat M, Escoffre-Barbe M, Blum S, Baumann M, Banos A, Straetmans N, Gallego-Hernanz MP, Chalandon Y, Graux C, Soulier J, Leguay T, Hunault M, Huguet F, Lhéritier V, Dombret H, Boissel N, Clappier E
Blood 2023 Nov 23;142(21):1806-1817. doi: 10.1182/blood.2023021501. PMID: 37595275
Results of NOPHO ALL2008 treatment for patients aged 1-45 years with acute lymphoblastic leukemia.
Toft N, Birgens H, Abrahamsson J, Griškevičius L, Hallböök H, Heyman M, Klausen TW, Jónsson ÓG, Palk K, Pruunsild K, Quist-Paulsen P, Vaitkeviciene G, Vettenranta K, Åsberg A, Frandsen TL, Marquart HV, Madsen HO, Norén-Nyström U, Schmiegelow K
Leukemia 2018 Mar;32(3):606-615. Epub 2017 Aug 18 doi: 10.1038/leu.2017.265. PMID: 28819280
MLL leukemia and future treatment strategies.
Marschalek R
Arch Pharm (Weinheim) 2015 Apr;348(4):221-8. Epub 2015 Mar 4 doi: 10.1002/ardp.201400449. PMID: 25740345

Recent clinical studies

Etiology

Genetic alterations and MRD refine risk assessment for KMT2A-rearranged B-cell precursor ALL in adults: a GRAALL study.
Kim R, Bergugnat H, Pastoret C, Pasquier F, Raffoux E, Larcher L, Passet M, Grardel N, Delabesse E, Kubetzko S, Caye-Eude A, Meyer C, Marschalek R, Lafage-Pochitaloff M, Thiebaut-Bertrand A, Balsat M, Escoffre-Barbe M, Blum S, Baumann M, Banos A, Straetmans N, Gallego-Hernanz MP, Chalandon Y, Graux C, Soulier J, Leguay T, Hunault M, Huguet F, Lhéritier V, Dombret H, Boissel N, Clappier E
Blood 2023 Nov 23;142(21):1806-1817. doi: 10.1182/blood.2023021501. PMID: 37595275
Single-cell multiomics reveals increased plasticity, resistant populations, and stem-cell-like blasts in KMT2A-rearranged leukemia.
Chen C, Yu W, Alikarami F, Qiu Q, Chen CH, Flournoy J, Gao P, Uzun Y, Fang L, Davenport JW, Hu Y, Zhu Q, Wang K, Libbrecht C, Felmeister A, Rozich I, Ding YY, Hunger SP, Felix CA, Wu H, Brown PA, Guest EM, Barrett DM, Bernt KM, Tan K
Blood 2022 Apr 7;139(14):2198-2211. doi: 10.1182/blood.2021013442. PMID: 34864916Free PMC Article
MLL-Rearranged Acute Lymphoblastic Leukemia.
El Chaer F, Keng M, Ballen KK
Curr Hematol Malig Rep 2020 Apr;15(2):83-89. doi: 10.1007/s11899-020-00582-5. PMID: 32350732
Results of NOPHO ALL2008 treatment for patients aged 1-45 years with acute lymphoblastic leukemia.
Toft N, Birgens H, Abrahamsson J, Griškevičius L, Hallböök H, Heyman M, Klausen TW, Jónsson ÓG, Palk K, Pruunsild K, Quist-Paulsen P, Vaitkeviciene G, Vettenranta K, Åsberg A, Frandsen TL, Marquart HV, Madsen HO, Norén-Nyström U, Schmiegelow K
Leukemia 2018 Mar;32(3):606-615. Epub 2017 Aug 18 doi: 10.1038/leu.2017.265. PMID: 28819280
The MLL recombinome of acute leukemias in 2017.
Meyer C, Burmeister T, Gröger D, Tsaur G, Fechina L, Renneville A, Sutton R, Venn NC, Emerenciano M, Pombo-de-Oliveira MS, Barbieri Blunck C, Almeida Lopes B, Zuna J, Trka J, Ballerini P, Lapillonne H, De Braekeleer M, Cazzaniga G, Corral Abascal L, van der Velden VHJ, Delabesse E, Park TS, Oh SH, Silva MLM, Lund-Aho T, Juvonen V, Moore AS, Heidenreich O, Vormoor J, Zerkalenkova E, Olshanskaya Y, Bueno C, Menendez P, Teigler-Schlegel A, Zur Stadt U, Lentes J, Göhring G, Kustanovich A, Aleinikova O, Schäfer BW, Kubetzko S, Madsen HO, Gruhn B, Duarte X, Gameiro P, Lippert E, Bidet A, Cayuela JM, Clappier E, Alonso CN, Zwaan CM, van den Heuvel-Eibrink MM, Izraeli S, Trakhtenbrot L, Archer P, Hancock J, Möricke A, Alten J, Schrappe M, Stanulla M, Strehl S, Attarbaschi A, Dworzak M, Haas OA, Panzer-Grümayer R, Sedék L, Szczepański T, Caye A, Suarez L, Cavé H, Marschalek R
Leukemia 2018 Feb;32(2):273-284. Epub 2017 Jul 13 doi: 10.1038/leu.2017.213. PMID: 28701730Free PMC Article

Diagnosis

Blinatumomab Added to Chemotherapy in Infant Lymphoblastic Leukemia.
van der Sluis IM, de Lorenzo P, Kotecha RS, Attarbaschi A, Escherich G, Nysom K, Stary J, Ferster A, Brethon B, Locatelli F, Schrappe M, Scholte-van Houtem PE, Valsecchi MG, Pieters R
N Engl J Med 2023 Apr 27;388(17):1572-1581. doi: 10.1056/NEJMoa2214171. PMID: 37099340
Single-cell multiomics reveals increased plasticity, resistant populations, and stem-cell-like blasts in KMT2A-rearranged leukemia.
Chen C, Yu W, Alikarami F, Qiu Q, Chen CH, Flournoy J, Gao P, Uzun Y, Fang L, Davenport JW, Hu Y, Zhu Q, Wang K, Libbrecht C, Felmeister A, Rozich I, Ding YY, Hunger SP, Felix CA, Wu H, Brown PA, Guest EM, Barrett DM, Bernt KM, Tan K
Blood 2022 Apr 7;139(14):2198-2211. doi: 10.1182/blood.2021013442. PMID: 34864916Free PMC Article
Acute Leukemia in Infants.
Ibrahimova A, Pommert L, Breese EH
Curr Oncol Rep 2021 Feb 12;23(3):27. doi: 10.1007/s11912-021-01021-1. PMID: 33580326
MLL-Rearranged Acute Lymphoblastic Leukemia.
El Chaer F, Keng M, Ballen KK
Curr Hematol Malig Rep 2020 Apr;15(2):83-89. doi: 10.1007/s11899-020-00582-5. PMID: 32350732
Results of NOPHO ALL2008 treatment for patients aged 1-45 years with acute lymphoblastic leukemia.
Toft N, Birgens H, Abrahamsson J, Griškevičius L, Hallböök H, Heyman M, Klausen TW, Jónsson ÓG, Palk K, Pruunsild K, Quist-Paulsen P, Vaitkeviciene G, Vettenranta K, Åsberg A, Frandsen TL, Marquart HV, Madsen HO, Norén-Nyström U, Schmiegelow K
Leukemia 2018 Mar;32(3):606-615. Epub 2017 Aug 18 doi: 10.1038/leu.2017.265. PMID: 28819280

Therapy

Blinatumomab Added to Chemotherapy in Infant Lymphoblastic Leukemia.
van der Sluis IM, de Lorenzo P, Kotecha RS, Attarbaschi A, Escherich G, Nysom K, Stary J, Ferster A, Brethon B, Locatelli F, Schrappe M, Scholte-van Houtem PE, Valsecchi MG, Pieters R
N Engl J Med 2023 Apr 27;388(17):1572-1581. doi: 10.1056/NEJMoa2214171. PMID: 37099340
Single-cell multiomics reveals increased plasticity, resistant populations, and stem-cell-like blasts in KMT2A-rearranged leukemia.
Chen C, Yu W, Alikarami F, Qiu Q, Chen CH, Flournoy J, Gao P, Uzun Y, Fang L, Davenport JW, Hu Y, Zhu Q, Wang K, Libbrecht C, Felmeister A, Rozich I, Ding YY, Hunger SP, Felix CA, Wu H, Brown PA, Guest EM, Barrett DM, Bernt KM, Tan K
Blood 2022 Apr 7;139(14):2198-2211. doi: 10.1182/blood.2021013442. PMID: 34864916Free PMC Article
MLL-Rearranged Acute Lymphoblastic Leukemia.
El Chaer F, Keng M, Ballen KK
Curr Hematol Malig Rep 2020 Apr;15(2):83-89. doi: 10.1007/s11899-020-00582-5. PMID: 32350732
Outcome of Infants Younger Than 1 Year With Acute Lymphoblastic Leukemia Treated With the Interfant-06 Protocol: Results From an International Phase III Randomized Study.
Pieters R, De Lorenzo P, Ancliffe P, Aversa LA, Brethon B, Biondi A, Campbell M, Escherich G, Ferster A, Gardner RA, Kotecha RS, Lausen B, Li CK, Locatelli F, Attarbaschi A, Peters C, Rubnitz JE, Silverman LB, Stary J, Szczepanski T, Vora A, Schrappe M, Valsecchi MG
J Clin Oncol 2019 Sep 1;37(25):2246-2256. Epub 2019 Jul 8 doi: 10.1200/JCO.19.00261. PMID: 31283407
Results of NOPHO ALL2008 treatment for patients aged 1-45 years with acute lymphoblastic leukemia.
Toft N, Birgens H, Abrahamsson J, Griškevičius L, Hallböök H, Heyman M, Klausen TW, Jónsson ÓG, Palk K, Pruunsild K, Quist-Paulsen P, Vaitkeviciene G, Vettenranta K, Åsberg A, Frandsen TL, Marquart HV, Madsen HO, Norén-Nyström U, Schmiegelow K
Leukemia 2018 Mar;32(3):606-615. Epub 2017 Aug 18 doi: 10.1038/leu.2017.265. PMID: 28819280

Prognosis

Genetic alterations and MRD refine risk assessment for KMT2A-rearranged B-cell precursor ALL in adults: a GRAALL study.
Kim R, Bergugnat H, Pastoret C, Pasquier F, Raffoux E, Larcher L, Passet M, Grardel N, Delabesse E, Kubetzko S, Caye-Eude A, Meyer C, Marschalek R, Lafage-Pochitaloff M, Thiebaut-Bertrand A, Balsat M, Escoffre-Barbe M, Blum S, Baumann M, Banos A, Straetmans N, Gallego-Hernanz MP, Chalandon Y, Graux C, Soulier J, Leguay T, Hunault M, Huguet F, Lhéritier V, Dombret H, Boissel N, Clappier E
Blood 2023 Nov 23;142(21):1806-1817. doi: 10.1182/blood.2023021501. PMID: 37595275
Blinatumomab Added to Chemotherapy in Infant Lymphoblastic Leukemia.
van der Sluis IM, de Lorenzo P, Kotecha RS, Attarbaschi A, Escherich G, Nysom K, Stary J, Ferster A, Brethon B, Locatelli F, Schrappe M, Scholte-van Houtem PE, Valsecchi MG, Pieters R
N Engl J Med 2023 Apr 27;388(17):1572-1581. doi: 10.1056/NEJMoa2214171. PMID: 37099340
Infantile B-lymphoblastic leukemia: a case series and review of the literature.
Mansoor N, Imran S, Maqsood S, Pasha S, Jabbar N
J Int Med Res 2023 Apr;51(4):3000605231167789. doi: 10.1177/03000605231167789. PMID: 37066443Free PMC Article
MLL-Rearranged Acute Lymphoblastic Leukemia.
El Chaer F, Keng M, Ballen KK
Curr Hematol Malig Rep 2020 Apr;15(2):83-89. doi: 10.1007/s11899-020-00582-5. PMID: 32350732
Outcome of Infants Younger Than 1 Year With Acute Lymphoblastic Leukemia Treated With the Interfant-06 Protocol: Results From an International Phase III Randomized Study.
Pieters R, De Lorenzo P, Ancliffe P, Aversa LA, Brethon B, Biondi A, Campbell M, Escherich G, Ferster A, Gardner RA, Kotecha RS, Lausen B, Li CK, Locatelli F, Attarbaschi A, Peters C, Rubnitz JE, Silverman LB, Stary J, Szczepanski T, Vora A, Schrappe M, Valsecchi MG
J Clin Oncol 2019 Sep 1;37(25):2246-2256. Epub 2019 Jul 8 doi: 10.1200/JCO.19.00261. PMID: 31283407

Clinical prediction guides

Classification and genetics of pediatric B-other acute lymphoblastic leukemia by targeted RNA sequencing.
Migita NA, Jotta PY, Nascimento NPD, Vasconcelos VS, Centoducatte GL, Massirer KB, Azevedo AC, Brandalise SR, Yunes JA
Blood Adv 2023 Jul 11;7(13):2957-2971. doi: 10.1182/bloodadvances.2022009179. PMID: 36848637Free PMC Article
Multi-omics analysis defines highly refractory RAS burdened immature subgroup of infant acute lymphoblastic leukemia.
Isobe T, Takagi M, Sato-Otsubo A, Nishimura A, Nagae G, Yamagishi C, Tamura M, Tanaka Y, Asada S, Takeda R, Tsuchiya A, Wang X, Yoshida K, Nannya Y, Ueno H, Akazawa R, Kato I, Mikami T, Watanabe K, Sekiguchi M, Seki M, Kimura S, Hiwatari M, Kato M, Fukuda S, Tatsuno K, Tsutsumi S, Kanai A, Inaba T, Shiozawa Y, Shiraishi Y, Chiba K, Tanaka H, Kotecha RS, Cruickshank MN, Ishikawa F, Morio T, Eguchi M, Deguchi T, Kiyokawa N, Arakawa Y, Koh K, Aoki Y, Ishihara T, Tomizawa D, Miyamura T, Ishii E, Mizutani S, Wilson NK, Göttgens B, Miyano S, Kitamura T, Goyama S, Yokoyama A, Aburatani H, Ogawa S, Takita J
Nat Commun 2022 Aug 30;13(1):4501. doi: 10.1038/s41467-022-32266-4. PMID: 36042201Free PMC Article
Single-cell multiomics reveals increased plasticity, resistant populations, and stem-cell-like blasts in KMT2A-rearranged leukemia.
Chen C, Yu W, Alikarami F, Qiu Q, Chen CH, Flournoy J, Gao P, Uzun Y, Fang L, Davenport JW, Hu Y, Zhu Q, Wang K, Libbrecht C, Felmeister A, Rozich I, Ding YY, Hunger SP, Felix CA, Wu H, Brown PA, Guest EM, Barrett DM, Bernt KM, Tan K
Blood 2022 Apr 7;139(14):2198-2211. doi: 10.1182/blood.2021013442. PMID: 34864916Free PMC Article
The MLL recombinome of acute leukemias in 2017.
Meyer C, Burmeister T, Gröger D, Tsaur G, Fechina L, Renneville A, Sutton R, Venn NC, Emerenciano M, Pombo-de-Oliveira MS, Barbieri Blunck C, Almeida Lopes B, Zuna J, Trka J, Ballerini P, Lapillonne H, De Braekeleer M, Cazzaniga G, Corral Abascal L, van der Velden VHJ, Delabesse E, Park TS, Oh SH, Silva MLM, Lund-Aho T, Juvonen V, Moore AS, Heidenreich O, Vormoor J, Zerkalenkova E, Olshanskaya Y, Bueno C, Menendez P, Teigler-Schlegel A, Zur Stadt U, Lentes J, Göhring G, Kustanovich A, Aleinikova O, Schäfer BW, Kubetzko S, Madsen HO, Gruhn B, Duarte X, Gameiro P, Lippert E, Bidet A, Cayuela JM, Clappier E, Alonso CN, Zwaan CM, van den Heuvel-Eibrink MM, Izraeli S, Trakhtenbrot L, Archer P, Hancock J, Möricke A, Alten J, Schrappe M, Stanulla M, Strehl S, Attarbaschi A, Dworzak M, Haas OA, Panzer-Grümayer R, Sedék L, Szczepański T, Caye A, Suarez L, Cavé H, Marschalek R
Leukemia 2018 Feb;32(2):273-284. Epub 2017 Jul 13 doi: 10.1038/leu.2017.213. PMID: 28701730Free PMC Article
Updates in the biology and therapy for infant acute lymphoblastic leukemia.
Guest EM, Stam RW
Curr Opin Pediatr 2017 Feb;29(1):20-26. doi: 10.1097/MOP.0000000000000437. PMID: 27841777

Recent systematic reviews

Is acute lymphoblastic leukemia with mature B-cell phenotype and KMT2A rearrangements a new entity? A systematic review and meta-analysis.
Hidalgo-Gómez G, Palacio-Garcia C, Gallur L, Blanco A, Tazón-Vega B, Saumell S, Martínez N, Murillo L, Murciano T, Velasco P, Bosch F, Diaz-Heredia C, Ortega M
Leuk Lymphoma 2021 Sep;62(9):2202-2210. Epub 2021 Apr 7 doi: 10.1080/10428194.2021.1907375. PMID: 33827367

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