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Blast phase chronic myelogenous leukemia, BCR-ABL1 positive

MedGen UID:
2281
•Concept ID:
C0005699
•
Neoplastic Process
Synonyms: Blast Crises; Blast Crisis; Blast Phase; Blast Phases; Crises, Blast; Crisis, Blast; Phase, Blast; Phases, Blast
SNOMED CT: Blastic phase chronic myeloid leukemia (413656006); Blast crisis stage of chronic myeloid leukemia (413656006); Blast crisis (278180008)
 
Monarch Initiative: MONDO:0006115

Definition

An advanced phase of chronic myeloid leukemia. It is characterized by: 1. the presence of blasts in the peripheral blood or bone marrow that are at least 20% of the peripheral blood white cells or of the nucleated cells in the bone marrow respectively, or 2. an extramedullary proliferation of blasts, and/or 3. when there are large aggregates and clusters of blasts in the bone marrow biopsy specimen (adapted from WHO, 2001). [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBlast phase chronic myelogenous leukemia, BCR-ABL1 positive

Professional guidelines

PubMed

Chronic Myeloid Leukemia, Version 2.2024, NCCN Clinical Practice Guidelines in Oncology.
Shah NP, Bhatia R, Altman JK, Amaya M, Begna KH, Berman E, Chan O, Clements J, Collins RH, Curtin PT, DeAngelo DJ, Drazer M, Maness L, Metheny L, Mohan S, Moore JO, Oehler V, Pratz K, Pusic I, Rose MG, Shomali W, Smith BD, Styler M, Talpaz M, Tanaka TN, Tantravahi S, Thompson J, Tsai S, Vaughn J, Welborn J, Yang DT, Sundar H, Gregory K
J Natl Compr Canc Netw 2024 Feb;22(1):43-69. doi: 10.6004/jnccn.2024.0007. PMID: 38394770
Blast phase myeloproliferative neoplasm: contemporary review and 2024 treatment algorithm.
Tefferi A, Alkhateeb H, Gangat N
Blood Cancer J 2023 Jul 18;13(1):108. doi: 10.1038/s41408-023-00878-8. PMID: 37460550Free PMC Article
Pathogenesis and management of accelerated and blast phases of chronic myeloid leukemia.
Senapati J, Jabbour E, Kantarjian H, Short NJ
Leukemia 2023 Jan;37(1):5-17. Epub 2022 Oct 29 doi: 10.1038/s41375-022-01736-5. PMID: 36309558

Recent clinical studies

Etiology

Accelerated-phase CML: de novo and transformed.
Shanmuganathan N, Hughes TP
Hematology Am Soc Hematol Educ Program 2023 Dec 8;2023(1):459-468. doi: 10.1182/hematology.2023000446. PMID: 38066863Free PMC Article
Blast phase myeloproliferative neoplasm: contemporary review and 2024 treatment algorithm.
Tefferi A, Alkhateeb H, Gangat N
Blood Cancer J 2023 Jul 18;13(1):108. doi: 10.1038/s41408-023-00878-8. PMID: 37460550Free PMC Article
Treatment of blast phase chronic myeloid leukaemia: A rare and challenging entity.
Copland M
Br J Haematol 2022 Dec;199(5):665-678. Epub 2022 Jul 22 doi: 10.1111/bjh.18370. PMID: 35866251Free PMC Article
Accelerated Phase of Myeloproliferative Neoplasms.
Shahin OA, Chifotides HT, Bose P, Masarova L, Verstovsek S
Acta Haematol 2021;144(5):484-499. Epub 2021 Apr 21 doi: 10.1159/000512929. PMID: 33882481Free PMC Article
Chronic Myeloid Leukemia: Modern therapies, current challenges and future directions.
Osman AEG, Deininger MW
Blood Rev 2021 Sep;49:100825. Epub 2021 Mar 16 doi: 10.1016/j.blre.2021.100825. PMID: 33773846Free PMC Article

Diagnosis

Chronic Myeloid Leukemia, Version 2.2024, NCCN Clinical Practice Guidelines in Oncology.
Shah NP, Bhatia R, Altman JK, Amaya M, Begna KH, Berman E, Chan O, Clements J, Collins RH, Curtin PT, DeAngelo DJ, Drazer M, Maness L, Metheny L, Mohan S, Moore JO, Oehler V, Pratz K, Pusic I, Rose MG, Shomali W, Smith BD, Styler M, Talpaz M, Tanaka TN, Tantravahi S, Thompson J, Tsai S, Vaughn J, Welborn J, Yang DT, Sundar H, Gregory K
J Natl Compr Canc Netw 2024 Feb;22(1):43-69. doi: 10.6004/jnccn.2024.0007. PMID: 38394770
Blast phase myeloproliferative neoplasm: contemporary review and 2024 treatment algorithm.
Tefferi A, Alkhateeb H, Gangat N
Blood Cancer J 2023 Jul 18;13(1):108. doi: 10.1038/s41408-023-00878-8. PMID: 37460550Free PMC Article
Ponatinib and blinatumomab for Philadelphia chromosome-positive acute lymphoblastic leukaemia: a US, single-centre, single-arm, phase 2 trial.
Jabbour E, Short NJ, Jain N, Huang X, Montalban-Bravo G, Banerjee P, Rezvani K, Jiang X, Kim KH, Kanagal-Shamanna R, Khoury JD, Patel K, Kadia TM, Daver N, Chien K, Alvarado Y, Garcia-Manero G, Issa GC, Haddad FG, Kwari M, Thankachan J, Delumpa R, Macaron W, Garris R, Konopleva M, Ravandi F, Kantarjian H
Lancet Haematol 2023 Jan;10(1):e24-e34. Epub 2022 Nov 16 doi: 10.1016/S2352-3026(22)00319-2. PMID: 36402146
Pathogenesis and management of accelerated and blast phases of chronic myeloid leukemia.
Senapati J, Jabbour E, Kantarjian H, Short NJ
Leukemia 2023 Jan;37(1):5-17. Epub 2022 Oct 29 doi: 10.1038/s41375-022-01736-5. PMID: 36309558
T-cell acute lymphoblastic leukemia.
Raetz EA, Teachey DT
Hematology Am Soc Hematol Educ Program 2016 Dec 2;2016(1):580-588. doi: 10.1182/asheducation-2016.1.580. PMID: 27913532Free PMC Article

Therapy

Chronic Myeloid Leukemia, Version 2.2024, NCCN Clinical Practice Guidelines in Oncology.
Shah NP, Bhatia R, Altman JK, Amaya M, Begna KH, Berman E, Chan O, Clements J, Collins RH, Curtin PT, DeAngelo DJ, Drazer M, Maness L, Metheny L, Mohan S, Moore JO, Oehler V, Pratz K, Pusic I, Rose MG, Shomali W, Smith BD, Styler M, Talpaz M, Tanaka TN, Tantravahi S, Thompson J, Tsai S, Vaughn J, Welborn J, Yang DT, Sundar H, Gregory K
J Natl Compr Canc Netw 2024 Feb;22(1):43-69. doi: 10.6004/jnccn.2024.0007. PMID: 38394770
Ponatinib and blinatumomab for Philadelphia chromosome-positive acute lymphoblastic leukaemia: a US, single-centre, single-arm, phase 2 trial.
Jabbour E, Short NJ, Jain N, Huang X, Montalban-Bravo G, Banerjee P, Rezvani K, Jiang X, Kim KH, Kanagal-Shamanna R, Khoury JD, Patel K, Kadia TM, Daver N, Chien K, Alvarado Y, Garcia-Manero G, Issa GC, Haddad FG, Kwari M, Thankachan J, Delumpa R, Macaron W, Garris R, Konopleva M, Ravandi F, Kantarjian H
Lancet Haematol 2023 Jan;10(1):e24-e34. Epub 2022 Nov 16 doi: 10.1016/S2352-3026(22)00319-2. PMID: 36402146
Pathogenesis and management of accelerated and blast phases of chronic myeloid leukemia.
Senapati J, Jabbour E, Kantarjian H, Short NJ
Leukemia 2023 Jan;37(1):5-17. Epub 2022 Oct 29 doi: 10.1038/s41375-022-01736-5. PMID: 36309558
Treatment of blast phase chronic myeloid leukaemia: A rare and challenging entity.
Copland M
Br J Haematol 2022 Dec;199(5):665-678. Epub 2022 Jul 22 doi: 10.1111/bjh.18370. PMID: 35866251Free PMC Article
Management of advanced-phase chronic myeloid leukemia.
DeFilipp Z, Khoury HJ
Curr Hematol Malig Rep 2015 Jun;10(2):173-81. doi: 10.1007/s11899-015-0249-2. PMID: 25929768

Prognosis

Blast phase myeloproliferative neoplasm: contemporary review and 2024 treatment algorithm.
Tefferi A, Alkhateeb H, Gangat N
Blood Cancer J 2023 Jul 18;13(1):108. doi: 10.1038/s41408-023-00878-8. PMID: 37460550Free PMC Article
Management of children and adolescents with chronic myeloid leukemia in blast phase: International pediatric CML expert panel recommendations.
Sembill S, Ampatzidou M, Chaudhury S, Dworzak M, Kalwak K, Karow A, Kiani A, Krumbholz M, Luesink M, Naumann-Bartsch N, De Moerloose B, Osborn M, Schultz KR, Sedlacek P, Giona F, Zwaan CM, Shimada H, Versluijs B, Millot F, Hijiya N, Suttorp M, Metzler M
Leukemia 2023 Mar;37(3):505-517. Epub 2023 Jan 27 doi: 10.1038/s41375-023-01822-2. PMID: 36707619Free PMC Article
Pathogenesis and management of accelerated and blast phases of chronic myeloid leukemia.
Senapati J, Jabbour E, Kantarjian H, Short NJ
Leukemia 2023 Jan;37(1):5-17. Epub 2022 Oct 29 doi: 10.1038/s41375-022-01736-5. PMID: 36309558
Accelerated Phase of Myeloproliferative Neoplasms.
Shahin OA, Chifotides HT, Bose P, Masarova L, Verstovsek S
Acta Haematol 2021;144(5):484-499. Epub 2021 Apr 21 doi: 10.1159/000512929. PMID: 33882481Free PMC Article
T-cell acute lymphoblastic leukemia.
Raetz EA, Teachey DT
Hematology Am Soc Hematol Educ Program 2016 Dec 2;2016(1):580-588. doi: 10.1182/asheducation-2016.1.580. PMID: 27913532Free PMC Article

Clinical prediction guides

Accelerated-phase CML: de novo and transformed.
Shanmuganathan N, Hughes TP
Hematology Am Soc Hematol Educ Program 2023 Dec 8;2023(1):459-468. doi: 10.1182/hematology.2023000446. PMID: 38066863Free PMC Article
Ponatinib and blinatumomab for Philadelphia chromosome-positive acute lymphoblastic leukaemia: a US, single-centre, single-arm, phase 2 trial.
Jabbour E, Short NJ, Jain N, Huang X, Montalban-Bravo G, Banerjee P, Rezvani K, Jiang X, Kim KH, Kanagal-Shamanna R, Khoury JD, Patel K, Kadia TM, Daver N, Chien K, Alvarado Y, Garcia-Manero G, Issa GC, Haddad FG, Kwari M, Thankachan J, Delumpa R, Macaron W, Garris R, Konopleva M, Ravandi F, Kantarjian H
Lancet Haematol 2023 Jan;10(1):e24-e34. Epub 2022 Nov 16 doi: 10.1016/S2352-3026(22)00319-2. PMID: 36402146
Treatment of blast phase chronic myeloid leukaemia: A rare and challenging entity.
Copland M
Br J Haematol 2022 Dec;199(5):665-678. Epub 2022 Jul 22 doi: 10.1111/bjh.18370. PMID: 35866251Free PMC Article
Accelerated Phase of Myeloproliferative Neoplasms.
Shahin OA, Chifotides HT, Bose P, Masarova L, Verstovsek S
Acta Haematol 2021;144(5):484-499. Epub 2021 Apr 21 doi: 10.1159/000512929. PMID: 33882481Free PMC Article
T-cell acute lymphoblastic leukemia.
Raetz EA, Teachey DT
Hematology Am Soc Hematol Educ Program 2016 Dec 2;2016(1):580-588. doi: 10.1182/asheducation-2016.1.580. PMID: 27913532Free PMC Article

Recent systematic reviews

Efficacy and safety of combination therapies vs monotherapy of hypomethylating agents in accelerated or blast phase of Philadelphia negative myeloproliferative neoplasms: a systematic review and meta-analysis.
Chen J, Wang K, Xiao Z, Xu Z
Ann Med 2023 Dec;55(1):348-360. doi: 10.1080/07853890.2022.2164611. PMID: 36644935Free PMC Article
Management of chronic myeloid leukemia in myeloid blastic phase with novel therapies: a systematic literature review.
Yılmaz U, Bulan B, Belli Ç, Eşkazan AE
Expert Rev Hematol 2022 May;15(5):423-429. Epub 2022 May 30 doi: 10.1080/17474086.2022.2076669. PMID: 35536916
Systematic Review of Normal Subjects Harbouring BCR-ABL1 Fusion Gene.
Kuan JW, Su AT, Leong CF, Osato M, Sashida G
Acta Haematol 2020;143(2):96-111. Epub 2019 Aug 9 doi: 10.1159/000501146. PMID: 31401626
Comparative Effectiveness of Newer Tyrosine Kinase Inhibitors Versus Imatinib in the First-Line Treatment of Chronic-Phase Chronic Myeloid Leukemia Across Risk Groups: A Systematic Review and Meta-Analysis of Eight Randomized Trials.
Yun S, Vincelette ND, Segar JM, Dong Y, Shen Y, Kim DW, Abraham I
Clin Lymphoma Myeloma Leuk 2016 Jun;16(6):e85-94. Epub 2016 Mar 30 doi: 10.1016/j.clml.2016.03.003. PMID: 27101984
Dasatinib and nilotinib for imatinib-resistant or -intolerant chronic myeloid leukaemia: a systematic review and economic evaluation.
Rogers G, Hoyle M, Thompson Coon J, Moxham T, Liu Z, Pitt M, Stein K
Health Technol Assess 2012;16(22):1-410. doi: 10.3310/hta16220. PMID: 22551803Free PMC Article

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