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B Acute Lymphoblastic Leukemia, Philadelphia Chromosome Negative

MedGen UID:
927478
•Concept ID:
C4289946
•
Neoplastic Process
Synonyms: Acute Lymphoblastic Leukemia, Philadelphia Chromosome Negative; B-Cell Acute Lymphoblastic Leukemia, Philadelphia Chromosome Negative; Ph1-Negative Acute Lymphoblastic Leukemia; Ph1-Negative ALL; Philadelphia Chromosome Negative Acute Lymphoblastic Leukemia; Philadelphia Chromosome Negative ALL; Philadelphia-Negative Acute Lymphoblastic Leukemia; Philadelphia-Negative ALL; Precursor B Lymphoblastic Leukemia, Philadelphia Chromosome Negative; Precursor B-Lymphoblastic Leukemia, Philadelphia Chromosome Negative

Definition

A precursor B acute lymphoblastic leukemia (B-ALL) which does not have the cytogenetic abnormality t(9;22)(q34;q11.2). Most cases of ALL do not have this translocation. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVB Acute Lymphoblastic Leukemia, Philadelphia Chromosome Negative

Professional guidelines

PubMed

Approach to the Treatment of Philadelphia Chromosome-Negative B-cell ALL in Older Adults: Is Age Becoming just a Number?
Liu Y, Duong VH
Curr Hematol Malig Rep 2023 Jun;18(3):68-74. Epub 2023 Mar 6 doi: 10.1007/s11899-023-00691-x. PMID: 36877337
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
Blinatumomab for the treatment of acute lymphoblastic leukemia.
Kaplan JB, Grischenko M, Giles FJ
Invest New Drugs 2015 Dec;33(6):1271-9. Epub 2015 Sep 17 doi: 10.1007/s10637-015-0289-4. PMID: 26383529

Recent clinical studies

Etiology

The evolution of acute lymphoblastic leukemia research and therapy at MD Anderson over four decades.
Jabbour E, Short NJ, Jain N, Haddad FG, Welch MA, Ravandi F, Kantarjian H
J Hematol Oncol 2023 Mar 16;16(1):22. doi: 10.1186/s13045-023-01409-5. PMID: 36927623Free PMC Article
SWOG 1318: A Phase II Trial of Blinatumomab Followed by POMP Maintenance in Older Patients With Newly Diagnosed Philadelphia Chromosome-Negative B-Cell Acute Lymphoblastic Leukemia.
Advani AS, Moseley A, O'Dwyer KM, Wood BL, Fang M, Wieduwilt MJ, Aldoss I, Park JH, Klisovic RB, Baer MR, Stock W, Bhave RR, Othus M, Harvey RC, Willman CL, Litzow MR, Stone RM, Sharon E, Erba HP
J Clin Oncol 2022 May 10;40(14):1574-1582. Epub 2022 Feb 14 doi: 10.1200/JCO.21.01766. PMID: 35157496Free PMC Article
Precision medicine in acute lymphoblastic leukemia.
Pui CH
Front Med 2020 Dec;14(6):689-700. Epub 2020 Oct 19 doi: 10.1007/s11684-020-0759-8. PMID: 33074527Free PMC Article
Salvage Chemoimmunotherapy With Inotuzumab Ozogamicin Combined With Mini-Hyper-CVD for Patients With Relapsed or Refractory Philadelphia Chromosome-Negative Acute Lymphoblastic Leukemia: A Phase 2 Clinical Trial.
Jabbour E, Ravandi F, Kebriaei P, Huang X, Short NJ, Thomas D, Sasaki K, Rytting M, Jain N, Konopleva M, Garcia-Manero G, Champlin R, Marin D, Kadia T, Cortes J, Estrov Z, Takahashi K, Patel Y, Khouri MR, Jacob J, Garris R, O'Brien S, Kantarjian H
JAMA Oncol 2018 Feb 1;4(2):230-234. doi: 10.1001/jamaoncol.2017.2380. PMID: 28859185Free PMC Article
Chemoimmunotherapy with a modified hyper-CVAD and rituximab regimen improves outcome in de novo Philadelphia chromosome-negative precursor B-lineage acute lymphoblastic leukemia.
Thomas DA, O'Brien S, Faderl S, Garcia-Manero G, Ferrajoli A, Wierda W, Ravandi F, Verstovsek S, Jorgensen JL, Bueso-Ramos C, Andreeff M, Pierce S, Garris R, Keating MJ, Cortes J, Kantarjian HM
J Clin Oncol 2010 Aug 20;28(24):3880-9. Epub 2010 Jul 26 doi: 10.1200/JCO.2009.26.9456. PMID: 20660823Free PMC Article

Diagnosis

Mini-hyper-CVD plus inotuzumab ozogamicin, with or without blinatumomab, in the subgroup of older patients with newly diagnosed Philadelphia chromosome-negative B-cell acute lymphocytic leukaemia: long-term results of an open-label phase 2 trial.
Jabbour E, Short NJ, Senapati J, Jain N, Huang X, Daver N, DiNardo CD, Pemmaraju N, Wierda W, Garcia-Manero G, Montalban Bravo G, Sasaki K, Kadia TM, Khoury J, Wang SA, Haddad FG, Jacob J, Garris R, Ravandi F, Kantarjian HM
Lancet Haematol 2023 Jun;10(6):e433-e444. Epub 2023 May 12 doi: 10.1016/S2352-3026(23)00073-X. PMID: 37187201
Hyper-CVAD and sequential blinatumomab for newly diagnosed Philadelphia chromosome-negative B-cell acute lymphocytic leukaemia: a single-arm, single-centre, phase 2 trial.
Jabbour E, Short NJ, Jain N, Thompson PA, Kadia TM, Ferrajoli A, Huang X, Yilmaz M, Alvarado Y, Patel KP, Garcia-Manero G, Macaron W, Garris R, Konopleva M, Ravandi F, Kantarjian H
Lancet Haematol 2022 Dec;9(12):e878-e885. Epub 2022 Oct 22 doi: 10.1016/S2352-3026(22)00285-X. PMID: 36279879
SWOG 1318: A Phase II Trial of Blinatumomab Followed by POMP Maintenance in Older Patients With Newly Diagnosed Philadelphia Chromosome-Negative B-Cell Acute Lymphoblastic Leukemia.
Advani AS, Moseley A, O'Dwyer KM, Wood BL, Fang M, Wieduwilt MJ, Aldoss I, Park JH, Klisovic RB, Baer MR, Stock W, Bhave RR, Othus M, Harvey RC, Willman CL, Litzow MR, Stone RM, Sharon E, Erba HP
J Clin Oncol 2022 May 10;40(14):1574-1582. Epub 2022 Feb 14 doi: 10.1200/JCO.21.01766. PMID: 35157496Free PMC Article
Philadelphia chromosome negative B-cell acute lymphoblastic leukemia in older adults: Current treatment and novel therapies.
O'Dwyer KM, Liesveld JL
Best Pract Res Clin Haematol 2017 Sep;30(3):184-192. Epub 2017 Aug 3 doi: 10.1016/j.beha.2017.08.001. PMID: 29050691
Philadelphia Chromosome-like Acute Lymphoblastic Leukemia.
Pui CH, Roberts KG, Yang JJ, Mullighan CG
Clin Lymphoma Myeloma Leuk 2017 Aug;17(8):464-470. doi: 10.1016/j.clml.2017.03.299. PMID: 28842136Free PMC Article

Therapy

The evolution of acute lymphoblastic leukemia research and therapy at MD Anderson over four decades.
Jabbour E, Short NJ, Jain N, Haddad FG, Welch MA, Ravandi F, Kantarjian H
J Hematol Oncol 2023 Mar 16;16(1):22. doi: 10.1186/s13045-023-01409-5. PMID: 36927623Free PMC Article
Hyper-CVAD and sequential blinatumomab for newly diagnosed Philadelphia chromosome-negative B-cell acute lymphocytic leukaemia: a single-arm, single-centre, phase 2 trial.
Jabbour E, Short NJ, Jain N, Thompson PA, Kadia TM, Ferrajoli A, Huang X, Yilmaz M, Alvarado Y, Patel KP, Garcia-Manero G, Macaron W, Garris R, Konopleva M, Ravandi F, Kantarjian H
Lancet Haematol 2022 Dec;9(12):e878-e885. Epub 2022 Oct 22 doi: 10.1016/S2352-3026(22)00285-X. PMID: 36279879
SWOG 1318: A Phase II Trial of Blinatumomab Followed by POMP Maintenance in Older Patients With Newly Diagnosed Philadelphia Chromosome-Negative B-Cell Acute Lymphoblastic Leukemia.
Advani AS, Moseley A, O'Dwyer KM, Wood BL, Fang M, Wieduwilt MJ, Aldoss I, Park JH, Klisovic RB, Baer MR, Stock W, Bhave RR, Othus M, Harvey RC, Willman CL, Litzow MR, Stone RM, Sharon E, Erba HP
J Clin Oncol 2022 May 10;40(14):1574-1582. Epub 2022 Feb 14 doi: 10.1200/JCO.21.01766. PMID: 35157496Free PMC Article
Salvage Chemoimmunotherapy With Inotuzumab Ozogamicin Combined With Mini-Hyper-CVD for Patients With Relapsed or Refractory Philadelphia Chromosome-Negative Acute Lymphoblastic Leukemia: A Phase 2 Clinical Trial.
Jabbour E, Ravandi F, Kebriaei P, Huang X, Short NJ, Thomas D, Sasaki K, Rytting M, Jain N, Konopleva M, Garcia-Manero G, Champlin R, Marin D, Kadia T, Cortes J, Estrov Z, Takahashi K, Patel Y, Khouri MR, Jacob J, Garris R, O'Brien S, Kantarjian H
JAMA Oncol 2018 Feb 1;4(2):230-234. doi: 10.1001/jamaoncol.2017.2380. PMID: 28859185Free PMC Article
Safety and activity of blinatumomab for adult patients with relapsed or refractory B-precursor acute lymphoblastic leukaemia: a multicentre, single-arm, phase 2 study.
Topp MS, Gökbuget N, Stein AS, Zugmaier G, O'Brien S, Bargou RC, Dombret H, Fielding AK, Heffner L, Larson RA, Neumann S, Foà R, Litzow M, Ribera JM, Rambaldi A, Schiller G, Brüggemann M, Horst HA, Holland C, Jia C, Maniar T, Huber B, Nagorsen D, Forman SJ, Kantarjian HM
Lancet Oncol 2015 Jan;16(1):57-66. Epub 2014 Dec 16 doi: 10.1016/S1470-2045(14)71170-2. PMID: 25524800

Prognosis

Hyper-CVAD and sequential blinatumomab for newly diagnosed Philadelphia chromosome-negative B-cell acute lymphocytic leukaemia: a single-arm, single-centre, phase 2 trial.
Jabbour E, Short NJ, Jain N, Thompson PA, Kadia TM, Ferrajoli A, Huang X, Yilmaz M, Alvarado Y, Patel KP, Garcia-Manero G, Macaron W, Garris R, Konopleva M, Ravandi F, Kantarjian H
Lancet Haematol 2022 Dec;9(12):e878-e885. Epub 2022 Oct 22 doi: 10.1016/S2352-3026(22)00285-X. PMID: 36279879
Salvage Chemoimmunotherapy With Inotuzumab Ozogamicin Combined With Mini-Hyper-CVD for Patients With Relapsed or Refractory Philadelphia Chromosome-Negative Acute Lymphoblastic Leukemia: A Phase 2 Clinical Trial.
Jabbour E, Ravandi F, Kebriaei P, Huang X, Short NJ, Thomas D, Sasaki K, Rytting M, Jain N, Konopleva M, Garcia-Manero G, Champlin R, Marin D, Kadia T, Cortes J, Estrov Z, Takahashi K, Patel Y, Khouri MR, Jacob J, Garris R, O'Brien S, Kantarjian H
JAMA Oncol 2018 Feb 1;4(2):230-234. doi: 10.1001/jamaoncol.2017.2380. PMID: 28859185Free PMC Article
Association of Minimal Residual Disease With Clinical Outcome in Pediatric and Adult Acute Lymphoblastic Leukemia: A Meta-analysis.
Berry DA, Zhou S, Higley H, Mukundan L, Fu S, Reaman GH, Wood BL, Kelloff GJ, Jessup JM, Radich JP
JAMA Oncol 2017 Jul 13;3(7):e170580. doi: 10.1001/jamaoncol.2017.0580. PMID: 28494052Free PMC Article
Safety and activity of blinatumomab for adult patients with relapsed or refractory B-precursor acute lymphoblastic leukaemia: a multicentre, single-arm, phase 2 study.
Topp MS, Gökbuget N, Stein AS, Zugmaier G, O'Brien S, Bargou RC, Dombret H, Fielding AK, Heffner L, Larson RA, Neumann S, Foà R, Litzow M, Ribera JM, Rambaldi A, Schiller G, Brüggemann M, Horst HA, Holland C, Jia C, Maniar T, Huber B, Nagorsen D, Forman SJ, Kantarjian HM
Lancet Oncol 2015 Jan;16(1):57-66. Epub 2014 Dec 16 doi: 10.1016/S1470-2045(14)71170-2. PMID: 25524800
Chemoimmunotherapy with a modified hyper-CVAD and rituximab regimen improves outcome in de novo Philadelphia chromosome-negative precursor B-lineage acute lymphoblastic leukemia.
Thomas DA, O'Brien S, Faderl S, Garcia-Manero G, Ferrajoli A, Wierda W, Ravandi F, Verstovsek S, Jorgensen JL, Bueso-Ramos C, Andreeff M, Pierce S, Garris R, Keating MJ, Cortes J, Kantarjian HM
J Clin Oncol 2010 Aug 20;28(24):3880-9. Epub 2010 Jul 26 doi: 10.1200/JCO.2009.26.9456. PMID: 20660823Free PMC Article

Clinical prediction guides

Real-world use of blinatumomab in adult patients with B-cell acute lymphoblastic leukemia in clinical practice: results from the NEUF study.
Boissel N, Chiaretti S, Papayannidis C, Ribera JM, Bassan R, Sokolov AN, Alam N, Brescianini A, Pezzani I, Kreuzbauer G, Zugmaier G, Foà R, Rambaldi A
Blood Cancer J 2023 Jan 4;13(1):2. doi: 10.1038/s41408-022-00766-7. PMID: 36599847Free PMC Article
Blinatumomab is associated with favorable outcomes in patients with B-cell lineage acute lymphoblastic leukemia and positive measurable residual disease at a threshold of 10(-4) and higher.
Jabbour EJ, Short NJ, Jain N, Jammal N, Jorgensen J, Wang S, Wang X, Ohanian M, Alvarado Y, Kadia T, Sasaki K, Garris R, Garcia-Manero G, Ravandi F, Kantarjian HM
Am J Hematol 2022 Sep;97(9):1135-1141. Epub 2022 Jun 24 doi: 10.1002/ajh.26634. PMID: 35713551
Biomarkers associated with blinatumomab outcomes in acute lymphoblastic leukemia.
Wei AH, Ribera JM, Larson RA, Ritchie D, Ghobadi A, Chen Y, Anderson A, Dos Santos CE, Franklin J, Kantarjian H
Leukemia 2021 Aug;35(8):2220-2231. Epub 2021 Feb 4 doi: 10.1038/s41375-020-01089-x. PMID: 33542479Free PMC Article
Intensified Therapy of Acute Lymphoblastic Leukemia in Adults: Report of the Randomized GRAALL-2005 Clinical Trial.
Huguet F, Chevret S, Leguay T, Thomas X, Boissel N, Escoffre-Barbe M, Chevallier P, Hunault M, Vey N, Bonmati C, Lepretre S, Marolleau JP, Pabst T, Rousselot P, Buzyn A, Cahn JY, Lhéritier V, Béné MC, Asnafi V, Delabesse E, Macintyre E, Chalandon Y, Ifrah N, Dombret H; Group of Research on Adult ALL (GRAALL)
J Clin Oncol 2018 Aug 20;36(24):2514-2523. Epub 2018 Jun 4 doi: 10.1200/JCO.2017.76.8192. PMID: 29863974
Rituximab in B-Lineage Adult Acute Lymphoblastic Leukemia.
Maury S, Chevret S, Thomas X, Heim D, Leguay T, Huguet F, Chevallier P, Hunault M, Boissel N, Escoffre-Barbe M, Hess U, Vey N, Pignon JM, Braun T, Marolleau JP, Cahn JY, Chalandon Y, Lhéritier V, Beldjord K, Béné MC, Ifrah N, Dombret H; for GRAALL
N Engl J Med 2016 Sep 15;375(11):1044-53. doi: 10.1056/NEJMoa1605085. PMID: 27626518

Recent systematic reviews

A systematic literature review and meta-analysis of minimal residual disease as a prognostic indicator in adult B-cell acute lymphoblastic leukemia.
Bassan R, Brüggemann M, Radcliffe HS, Hartfield E, Kreuzbauer G, Wetten S
Haematologica 2019 Oct;104(10):2028-2039. Epub 2019 Mar 19 doi: 10.3324/haematol.2018.201053. PMID: 30890593Free PMC Article

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