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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 B-acute lymphoblastic leukemia (B-ALL) that does not have the cytogenetic abnormality t(9;22)(q34;q11.2). Most cases of B-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

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
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
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
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
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

Dose-Dense Mini-Hyper-CVD, Inotuzumab Ozogamicin and Blinatumomab Achieves Rapid MRD-Negativity in Philadelphia Chromosome-Negative B-cell Acute Lymphoblastic Leukemia.
Short NJ, Jabbour E, Jamison T, Paul S, Cuglievan B, McCall D, Gibson A, Jain N, Haddad FG, Nasr LF, Marx KR, Rausch C, Savoy JM, Garris R, Ravandi F, Kantarjian H
Clin Lymphoma Myeloma Leuk 2024 Apr;24(4):e168-e173. Epub 2023 Dec 30 doi: 10.1016/j.clml.2023.12.016. PMID: 38212207
Inotuzumab Ozogamicin as Induction Therapy for Patients Older Than 55 Years With Philadelphia Chromosome-Negative B-Precursor ALL.
Stelljes M, Raffel S, Alakel N, Wäsch R, Kondakci M, Scholl S, Rank A, Hänel M, Spriewald B, Hanoun M, Martin S, Schwab K, Serve H, Reiser L, Knaden J, Pfeifer H, Marx J, Sauer T, Berdel WE, Lenz G, Brüggemann M, Gökbuget N, Wethmar K
J Clin Oncol 2024 Jan 20;42(3):273-282. Epub 2023 Oct 26 doi: 10.1200/JCO.23.00546. PMID: 37883727
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
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

Therapy

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
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
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
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

Prognosis

Inotuzumab Ozogamicin as Induction Therapy for Patients Older Than 55 Years With Philadelphia Chromosome-Negative B-Precursor ALL.
Stelljes M, Raffel S, Alakel N, Wäsch R, Kondakci M, Scholl S, Rank A, Hänel M, Spriewald B, Hanoun M, Martin S, Schwab K, Serve H, Reiser L, Knaden J, Pfeifer H, Marx J, Sauer T, Berdel WE, Lenz G, Brüggemann M, Gökbuget N, Wethmar K
J Clin Oncol 2024 Jan 20;42(3):273-282. Epub 2023 Oct 26 doi: 10.1200/JCO.23.00546. PMID: 37883727
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
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
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

Dose-Dense Mini-Hyper-CVD, Inotuzumab Ozogamicin and Blinatumomab Achieves Rapid MRD-Negativity in Philadelphia Chromosome-Negative B-cell Acute Lymphoblastic Leukemia.
Short NJ, Jabbour E, Jamison T, Paul S, Cuglievan B, McCall D, Gibson A, Jain N, Haddad FG, Nasr LF, Marx KR, Rausch C, Savoy JM, Garris R, Ravandi F, Kantarjian H
Clin Lymphoma Myeloma Leuk 2024 Apr;24(4):e168-e173. Epub 2023 Dec 30 doi: 10.1016/j.clml.2023.12.016. PMID: 38212207
Pegaspargase-modified risk-oriented program for adult acute lymphoblastic leukemia: results of the GIMEMA LAL1913 trial.
Bassan R, Chiaretti S, Della Starza I, Spinelli O, Santoro A, Paoloni F, Messina M, Elia L, De Propris MS, Scattolin AM, Audisio E, Marbello L, Borlenghi E, Zappasodi P, Mauro E, Martinelli G, Mattei D, Fracchiolla N, Bocchia M, De Fabritiis P, Bonifacio M, Candoni A, Cassibba V, Di Bartolomeo P, Latte G, Trappolini S, Guarini A, Vitale A, Fazi P, Piciocchi A, Rambaldi A, Foà R
Blood Adv 2023 Aug 22;7(16):4448-4461. doi: 10.1182/bloodadvances.2022009596. PMID: 37276451Free 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
Immunotherapy in Tumors.
Kobold S, Duewell P, Schnurr M, Subklewe M, Rothenfusser S, Endres S
Dtsch Arztebl Int 2015 Nov 27;112(48):809-15. doi: 10.3238/arztebl.2015.0809. PMID: 26667979Free PMC Article

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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