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Ph-positive acute lymphoblastic leukemia

MedGen UID:
313581
•Concept ID:
C1709527
•
Neoplastic Process
Synonyms: Acute Lymphoblastic Leukemia, Philadelphia Chromosome Positive; B Acute Lymphoblastic Leukemia with BCR-ABL1 Fusion; B Acute Lymphoblastic Leukemia with BCR::ABL1 Fusion; B Acute Lymphoblastic Leukemia with t(9;22)(q34.1;q11.2); BCR-ABL1; B Acute Lymphoblastic Leukemia with t(9;22)(q34.1;q11.2); BCR::ABL1; B Acute Lymphoblastic Leukemia with t(9;22)(q34;q11.2); BCR-ABL1; B-Acute Lymphoblastic Leukemia with BCR-ABL1 Fusion; B-Acute Lymphoblastic Leukemia with BCR::ABL1 Fusion; B-Acute Lymphoblastic Leukemia with t(9;22)(q34.1;q11.2); BCR-ABL1; B-Acute Lymphoblastic Leukemia with t(9;22)(q34.1;q11.2); BCR::ABL1; B-Cell Acute Lymphoblastic Leukemia with t(9;22)(q34;q11.2); BCR-ABL1; Ph positive ALL; Ph+ acute lymphoblastic leukemia; Ph+ ALL; Ph-positive acute lymphoblastic leukaemia; Philadelphia chromosome-positive acute lymphoblastic leukemia; Philadelphia Positive Acute Lymphoblastic Leukemia; Philadelphia Positive Precursor Lymphoblastic Leukemia; Philadelphia-positive acute lymphoblastic leukaemia; Philadelphia-positive acute lymphoblastic leukemia
SNOMED CT: Ph+ acute lymphoblastic leukemia (426955004); Philadelphia chromosome-positive acute lymphoblastic leukemia (426955004); Ph positive ALL (425688002); Ph+ ALL (425688002); Ph+ acute lymphoblastic leukemia (425688002); Philadelphia chromosome-positive acute lymphoblastic leukemia (425688002); Ph positive ALL (426955004); Ph+ ALL (426955004)
 
HPO: HP:0004848

Definition

A subset of acute lymphoblastic leukemia that results from a reciprocal translocation between the ABL-1 oncogene and a breakpoint cluster region (BCR), resulting in a fusion gene, BCR-ABL, that encodes an oncogenic protein with constitutively active tyrosine kinase activity. [from HPO]

Conditions with this feature

Chronic myelogenous leukemia, BCR-ABL1 positive
MedGen UID:
75993
•Concept ID:
C0279543
•
Neoplastic Process
A myeloproliferative disorder characterized by increased proliferation of the granulocytic cell line without the loss of their capacity to differentiate.
See: Condition Record
Chronic myelogenous leukemia, BCR-ABL1 positive

Professional guidelines

PubMed

Management of Philadelphia chromosome positive acute lymphoblastic leukemia in the current era.
Badar T, Alkhateeb H, Aljurf M, Kharfan-Dabaja MA
Curr Res Transl Med 2023 Apr-Jun;71(2):103392. Epub 2023 Apr 23 doi: 10.1016/j.retram.2023.103392. PMID: 37121211
Treatment of Adults With Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia-From Intensive Chemotherapy Combinations to Chemotherapy-Free Regimens: A Review.
Jabbour E, Haddad FG, Short NJ, Kantarjian H
JAMA Oncol 2022 Sep 1;8(9):1340-1348. doi: 10.1001/jamaoncol.2022.2398. PMID: 35834222
Treatment of Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia.
Abou Dalle I, Jabbour E, Short NJ, Ravandi F
Curr Treat Options Oncol 2019 Jan 24;20(1):4. doi: 10.1007/s11864-019-0603-z. PMID: 30675645Free PMC Article

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Pediatric Acute Lymphoblastic Leukemia, 2024

Recent clinical studies

Etiology

Ponatinib-review of historical development, current status, and future research.
Kantarjian HM, Chifotides HT, Haddad FG, Short NJ, Loghavi S, Jabbour E
Am J Hematol 2024 Aug;99(8):1576-1585. Epub 2024 May 10 doi: 10.1002/ajh.27355. PMID: 38727135Free PMC Article
SOHO State of the Art Updates and Next Questions: Update on the Approach to Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia.
Haddad FG, Jabbour E, Short NJ, Jain N, Kantarjian H
Clin Lymphoma Myeloma Leuk 2024 May;24(5):271-276. Epub 2023 Dec 14 doi: 10.1016/j.clml.2023.12.007. PMID: 38185587
Treatment of Adults With Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia-From Intensive Chemotherapy Combinations to Chemotherapy-Free Regimens: A Review.
Jabbour E, Haddad FG, Short NJ, Kantarjian H
JAMA Oncol 2022 Sep 1;8(9):1340-1348. doi: 10.1001/jamaoncol.2022.2398. PMID: 35834222
Dasatinib-Blinatumomab for Ph-Positive Acute Lymphoblastic Leukemia in Adults.
Foà R, Bassan R, Vitale A, Elia L, Piciocchi A, Puzzolo MC, Canichella M, Viero P, Ferrara F, Lunghi M, Fabbiano F, Bonifacio M, Fracchiolla N, Di Bartolomeo P, Mancino A, De Propris MS, Vignetti M, Guarini A, Rambaldi A, Chiaretti S; GIMEMA Investigators
N Engl J Med 2020 Oct 22;383(17):1613-1623. doi: 10.1056/NEJMoa2016272. PMID: 33085860
Randomized study of reduced-intensity chemotherapy combined with imatinib in adults with Ph-positive acute lymphoblastic leukemia.
Chalandon Y, Thomas X, Hayette S, Cayuela JM, Abbal C, Huguet F, Raffoux E, Leguay T, Rousselot P, Lepretre S, Escoffre-Barbe M, Maury S, Berthon C, Tavernier E, Lambert JF, Lafage-Pochitaloff M, Lhéritier V, Chevret S, Ifrah N, Dombret H; Group for Research on Adult Acute Lymphoblastic Leukemia (GRAALL)
Blood 2015 Jun 11;125(24):3711-9. Epub 2015 Apr 15 doi: 10.1182/blood-2015-02-627935. PMID: 25878120

Diagnosis

SOHO State of the Art Updates and Next Questions: Update on the Approach to Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia.
Haddad FG, Jabbour E, Short NJ, Jain N, Kantarjian H
Clin Lymphoma Myeloma Leuk 2024 May;24(5):271-276. Epub 2023 Dec 14 doi: 10.1016/j.clml.2023.12.007. PMID: 38185587
The emerging use of chemotherapy-free regimens in adults with Philadelphia chromosome-positive acute lymphoblastic leukemia.
Sawyers JD, Jammal NJ, Short NJ, Kantarjian H, Jabbour EJ
Clin Adv Hematol Oncol 2023 Feb;21(2):68-75. PMID: 36780472
Treatment of Adults With Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia-From Intensive Chemotherapy Combinations to Chemotherapy-Free Regimens: A Review.
Jabbour E, Haddad FG, Short NJ, Kantarjian H
JAMA Oncol 2022 Sep 1;8(9):1340-1348. doi: 10.1001/jamaoncol.2022.2398. PMID: 35834222
Ponatinib, chemotherapy, and transplant in adults with Philadelphia chromosome-positive acute lymphoblastic leukemia.
Ribera JM, García-Calduch O, Ribera J, Montesinos P, Cano-Ferri I, Martínez P, Esteve J, Esteban D, García-Fortes M, Alonso N, González-Campos J, Bermúdez A, Torrent A, Genescà E, Mercadal S, Martínez-Lopez J, García-Sanz R
Blood Adv 2022 Sep 27;6(18):5395-5402. doi: 10.1182/bloodadvances.2022007764. PMID: 35675590Free PMC Article
Dasatinib-Blinatumomab for Ph-Positive Acute Lymphoblastic Leukemia in Adults.
Foà R, Bassan R, Vitale A, Elia L, Piciocchi A, Puzzolo MC, Canichella M, Viero P, Ferrara F, Lunghi M, Fabbiano F, Bonifacio M, Fracchiolla N, Di Bartolomeo P, Mancino A, De Propris MS, Vignetti M, Guarini A, Rambaldi A, Chiaretti S; GIMEMA Investigators
N Engl J Med 2020 Oct 22;383(17):1613-1623. doi: 10.1056/NEJMoa2016272. PMID: 33085860

Therapy

Treatment of Adults With Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia-From Intensive Chemotherapy Combinations to Chemotherapy-Free Regimens: A Review.
Jabbour E, Haddad FG, Short NJ, Kantarjian H
JAMA Oncol 2022 Sep 1;8(9):1340-1348. doi: 10.1001/jamaoncol.2022.2398. PMID: 35834222
Ponatinib, chemotherapy, and transplant in adults with Philadelphia chromosome-positive acute lymphoblastic leukemia.
Ribera JM, García-Calduch O, Ribera J, Montesinos P, Cano-Ferri I, Martínez P, Esteve J, Esteban D, García-Fortes M, Alonso N, González-Campos J, Bermúdez A, Torrent A, Genescà E, Mercadal S, Martínez-Lopez J, García-Sanz R
Blood Adv 2022 Sep 27;6(18):5395-5402. doi: 10.1182/bloodadvances.2022007764. PMID: 35675590Free PMC Article
Dasatinib-Blinatumomab for Ph-Positive Acute Lymphoblastic Leukemia in Adults.
Foà R, Bassan R, Vitale A, Elia L, Piciocchi A, Puzzolo MC, Canichella M, Viero P, Ferrara F, Lunghi M, Fabbiano F, Bonifacio M, Fracchiolla N, Di Bartolomeo P, Mancino A, De Propris MS, Vignetti M, Guarini A, Rambaldi A, Chiaretti S; GIMEMA Investigators
N Engl J Med 2020 Oct 22;383(17):1613-1623. doi: 10.1056/NEJMoa2016272. PMID: 33085860
Management of adult Ph-positive acute lymphoblastic leukemia.
Chiaretti S, Foà R
Hematology Am Soc Hematol Educ Program 2015;2015:406-13. doi: 10.1182/asheducation-2015.1.406. PMID: 26637750
Randomized study of reduced-intensity chemotherapy combined with imatinib in adults with Ph-positive acute lymphoblastic leukemia.
Chalandon Y, Thomas X, Hayette S, Cayuela JM, Abbal C, Huguet F, Raffoux E, Leguay T, Rousselot P, Lepretre S, Escoffre-Barbe M, Maury S, Berthon C, Tavernier E, Lambert JF, Lafage-Pochitaloff M, Lhéritier V, Chevret S, Ifrah N, Dombret H; Group for Research on Adult Acute Lymphoblastic Leukemia (GRAALL)
Blood 2015 Jun 11;125(24):3711-9. Epub 2015 Apr 15 doi: 10.1182/blood-2015-02-627935. PMID: 25878120

Prognosis

SOHO State of the Art Updates and Next Questions: Update on the Approach to Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia.
Haddad FG, Jabbour E, Short NJ, Jain N, Kantarjian H
Clin Lymphoma Myeloma Leuk 2024 May;24(5):271-276. Epub 2023 Dec 14 doi: 10.1016/j.clml.2023.12.007. PMID: 38185587
Comparison of the Efficacy and Safety of Ponatinib and Dasatinib in Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia With Central Nervous System Relapse: A Retrospective Study.
Zhu Y, Zhu Y, Miao L, Jia T, Mao J, Xue L, Wang Y
Technol Cancer Res Treat 2023 Jan-Dec;22:15330338231165866. doi: 10.1177/15330338231165866. PMID: 36959735Free PMC Article
Clinical Impact of Drug Adherence of Tyrosine Kinase Inhibitors in Children with Ph-Positive Acute Lymphoblastic Leukemia.
Wang JX, Yang MM, Liu LP, Zhang HM, Wang MC, Chen YW, Zang XY, Hu F
Cancer Res Treat 2023 Jul;55(3):1023-1030. Epub 2023 Feb 6 doi: 10.4143/crt.2022.1618. PMID: 36758956Free PMC Article
Genetic correlates in patients with Philadelphia chromosome-positive acute lymphoblastic leukemia treated with Hyper-CVAD plus dasatinib or ponatinib.
Sasaki Y, Kantarjian HM, Short NJ, Wang F, Furudate K, Uryu H, Garris R, Jain N, Sasaki K, Ravandi F, Konopleva M, Garcia-Manero G, Little L, Gumbs C, Zhao L, Futreal PA, Takahashi K, Jabbour E
Leukemia 2022 May;36(5):1253-1260. Epub 2022 Feb 7 doi: 10.1038/s41375-021-01496-8. PMID: 35132195
The evolving role of chemotherapy and hematopoietic cell transplants in Ph-positive acute lymphoblastic leukemia in adults.
Litzow MR, Fielding AK, Luger SM, Paietta E, Ofran Y, Rowe JM, Goldstone AH, Tallman MS, Lazarus HM
Bone Marrow Transplant 2017 Dec;52(12):1592-1598. Epub 2017 Jun 5 doi: 10.1038/bmt.2017.110. PMID: 28581459

Clinical prediction guides

SOHO State of the Art Updates and Next Questions: Update on the Approach to Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia.
Haddad FG, Jabbour E, Short NJ, Jain N, Kantarjian H
Clin Lymphoma Myeloma Leuk 2024 May;24(5):271-276. Epub 2023 Dec 14 doi: 10.1016/j.clml.2023.12.007. PMID: 38185587
Treatment of Adults With Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia-From Intensive Chemotherapy Combinations to Chemotherapy-Free Regimens: A Review.
Jabbour E, Haddad FG, Short NJ, Kantarjian H
JAMA Oncol 2022 Sep 1;8(9):1340-1348. doi: 10.1001/jamaoncol.2022.2398. PMID: 35834222
Ponatinib, chemotherapy, and transplant in adults with Philadelphia chromosome-positive acute lymphoblastic leukemia.
Ribera JM, García-Calduch O, Ribera J, Montesinos P, Cano-Ferri I, Martínez P, Esteve J, Esteban D, García-Fortes M, Alonso N, González-Campos J, Bermúdez A, Torrent A, Genescà E, Mercadal S, Martínez-Lopez J, García-Sanz R
Blood Adv 2022 Sep 27;6(18):5395-5402. doi: 10.1182/bloodadvances.2022007764. PMID: 35675590Free PMC Article
Dasatinib-Blinatumomab for Ph-Positive Acute Lymphoblastic Leukemia in Adults.
Foà R, Bassan R, Vitale A, Elia L, Piciocchi A, Puzzolo MC, Canichella M, Viero P, Ferrara F, Lunghi M, Fabbiano F, Bonifacio M, Fracchiolla N, Di Bartolomeo P, Mancino A, De Propris MS, Vignetti M, Guarini A, Rambaldi A, Chiaretti S; GIMEMA Investigators
N Engl J Med 2020 Oct 22;383(17):1613-1623. doi: 10.1056/NEJMoa2016272. PMID: 33085860
Near-triploid Ph-positive leukemia.
Ohtaki K, Abe R, Tebbi CK, de los Santos R, Han T, Sandberg AA
Cancer Genet Cytogenet 1985 Oct;18(2):113-21. doi: 10.1016/0165-4608(85)90061-5. PMID: 3863695

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    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Pediatric Acute Lymphoblastic Leukemia, 2024

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