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Chronic Myeloid Leukemia, BCR-ABL1 Positive

MedGen UID:
714993
Concept ID:
C1292771
Neoplastic Process
Synonyms: BCR-ABL Positive Chronic Myelogenous Leukemia; Chronic Granulocytic Leukemia; Chronic Myelocytic Leukemia; Chronic Myelogenous Leukemia; Chronic Myelogenous Leukemia, BCR-ABL1 Positive; Chronic Myelogenous Leukemia, BCR::ABL1 Positive; Chronic Myelogenous Leukemias; Chronic Myeloid Leukemia; Chronic Myeloid Leukemia, BCR::ABL1 Positive; CML - Chronic Myelogenous Leukemia; Hematopoeitic - Chronic Myelocytic Leukemia (CML)

Definition

A chronic myeloproliferative neoplasm characterized by the expression of the BCR-ABL1 fusion gene. It presents with neutrophilic leukocytosis. It can appear at any age, but it mostly affects middle aged and older individuals. Patients usually present with fatigue, weight loss, anemia, night sweats, and splenomegaly. If untreated, it follows a biphasic or triphasic natural course; an initial indolent chronic phase which is followed by an accelerated phase, a blast phase, or both. Allogeneic stem cell transplantation and tyrosine kinase inhibitors delay disease progression and prolong overall survival. [from NCI]

Professional guidelines

PubMed

Ciftciler R, Haznedaroglu IC
Eur Rev Med Pharmacol Sci 2021 Dec;25(24):7787-7798. doi: 10.26355/eurrev_202112_27625. PMID: 34982440

Recent clinical studies

Etiology

Hochhaus A, Wang J, Kim DW, Kim DDH, Mayer J, Goh YT, le Coutre P, Takahashi N, Kim I, Etienne G, Andorsky D, Issa GC, Larson RA, Bombaci F, Kapoor S, McCulloch T, Malek K, Yau L, Ifrah S, Hoch M, Cortes JE, Hughes TP; ASC4FIRST Investigators
N Engl J Med 2024 Sep 12;391(10):885-898. Epub 2024 May 31 doi: 10.1056/NEJMoa2400858. PMID: 38820078
Senapati J, Sasaki K, Issa GC, Lipton JH, Radich JP, Jabbour E, Kantarjian HM
Blood Cancer J 2023 Apr 24;13(1):58. doi: 10.1038/s41408-023-00823-9. PMID: 37088793Free PMC Article
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
Hochhaus A, Baccarani M, Silver RT, Schiffer C, Apperley JF, Cervantes F, Clark RE, Cortes JE, Deininger MW, Guilhot F, Hjorth-Hansen H, Hughes TP, Janssen JJWM, Kantarjian HM, Kim DW, Larson RA, Lipton JH, Mahon FX, Mayer J, Nicolini F, Niederwieser D, Pane F, Radich JP, Rea D, Richter J, Rosti G, Rousselot P, Saglio G, Saußele S, Soverini S, Steegmann JL, Turkina A, Zaritskey A, Hehlmann R
Leukemia 2020 Apr;34(4):966-984. Epub 2020 Mar 3 doi: 10.1038/s41375-020-0776-2. PMID: 32127639Free PMC Article
Zhou T, Medeiros LJ, Hu S
Curr Hematol Malig Rep 2018 Dec;13(6):435-445. doi: 10.1007/s11899-018-0474-6. PMID: 30370478

Diagnosis

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
Jabbour E, Kantarjian H
Am J Hematol 2022 Sep;97(9):1236-1256. Epub 2022 Jul 6 doi: 10.1002/ajh.26642. PMID: 35751859
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
Hochhaus A, Baccarani M, Silver RT, Schiffer C, Apperley JF, Cervantes F, Clark RE, Cortes JE, Deininger MW, Guilhot F, Hjorth-Hansen H, Hughes TP, Janssen JJWM, Kantarjian HM, Kim DW, Larson RA, Lipton JH, Mahon FX, Mayer J, Nicolini F, Niederwieser D, Pane F, Radich JP, Rea D, Richter J, Rosti G, Rousselot P, Saglio G, Saußele S, Soverini S, Steegmann JL, Turkina A, Zaritskey A, Hehlmann R
Leukemia 2020 Apr;34(4):966-984. Epub 2020 Mar 3 doi: 10.1038/s41375-020-0776-2. PMID: 32127639Free PMC Article
Cortes JE, Jimenez CA, Mauro MJ, Geyer A, Pinilla-Ibarz J, Smith BD
Clin Lymphoma Myeloma Leuk 2017 Feb;17(2):78-82. Epub 2016 Sep 17 doi: 10.1016/j.clml.2016.09.012. PMID: 28082112

Therapy

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
Senapati J, Sasaki K, Issa GC, Lipton JH, Radich JP, Jabbour E, Kantarjian HM
Blood Cancer J 2023 Apr 24;13(1):58. doi: 10.1038/s41408-023-00823-9. PMID: 37088793Free PMC Article
Jabbour E, Kantarjian H
Am J Hematol 2022 Sep;97(9):1236-1256. Epub 2022 Jul 6 doi: 10.1002/ajh.26642. PMID: 35751859
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
Braun TP, Eide CA, Druker BJ
Cancer Cell 2020 Apr 13;37(4):530-542. doi: 10.1016/j.ccell.2020.03.006. PMID: 32289275Free PMC Article

Prognosis

Hochhaus A, Réa D, Boquimpani C, Minami Y, Cortes JE, Hughes TP, Apperley JF, Lomaia E, Voloshin S, Turkina A, Kim DW, Abdo A, Fogliatto LM, le Coutre P, Sasaki K, Kim DDH, Saussele S, Annunziata M, Chaudhri N, Chee L, García-Gutiérrez V, Kapoor S, Allepuz A, Quenet S, Bédoucha V, Mauro MJ
Leukemia 2023 Mar;37(3):617-626. Epub 2023 Jan 30 doi: 10.1038/s41375-023-01829-9. PMID: 36717654Free PMC Article
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
Jabbour E, Kantarjian H
Am J Hematol 2020 Jun;95(6):691-709. Epub 2020 Apr 10 doi: 10.1002/ajh.25792. PMID: 32239758
Hughes TP, Mauro MJ, Cortes JE, Minami H, Rea D, DeAngelo DJ, Breccia M, Goh YT, Talpaz M, Hochhaus A, le Coutre P, Ottmann O, Heinrich MC, Steegmann JL, Deininger MWN, Janssen JJWM, Mahon FX, Minami Y, Yeung D, Ross DM, Tallman MS, Park JH, Druker BJ, Hynds D, Duan Y, Meille C, Hourcade-Potelleret F, Vanasse KG, Lang F, Kim DW
N Engl J Med 2019 Dec 12;381(24):2315-2326. doi: 10.1056/NEJMoa1902328. PMID: 31826340Free PMC Article
Zhou T, Medeiros LJ, Hu S
Curr Hematol Malig Rep 2018 Dec;13(6):435-445. doi: 10.1007/s11899-018-0474-6. PMID: 30370478

Clinical prediction guides

Hochhaus A, Wang J, Kim DW, Kim DDH, Mayer J, Goh YT, le Coutre P, Takahashi N, Kim I, Etienne G, Andorsky D, Issa GC, Larson RA, Bombaci F, Kapoor S, McCulloch T, Malek K, Yau L, Ifrah S, Hoch M, Cortes JE, Hughes TP; ASC4FIRST Investigators
N Engl J Med 2024 Sep 12;391(10):885-898. Epub 2024 May 31 doi: 10.1056/NEJMoa2400858. PMID: 38820078
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
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
Hochhaus A, Baccarani M, Silver RT, Schiffer C, Apperley JF, Cervantes F, Clark RE, Cortes JE, Deininger MW, Guilhot F, Hjorth-Hansen H, Hughes TP, Janssen JJWM, Kantarjian HM, Kim DW, Larson RA, Lipton JH, Mahon FX, Mayer J, Nicolini F, Niederwieser D, Pane F, Radich JP, Rea D, Richter J, Rosti G, Rousselot P, Saglio G, Saußele S, Soverini S, Steegmann JL, Turkina A, Zaritskey A, Hehlmann R
Leukemia 2020 Apr;34(4):966-984. Epub 2020 Mar 3 doi: 10.1038/s41375-020-0776-2. PMID: 32127639Free PMC Article
Vetrie D, Helgason GV, Copland M
Nat Rev Cancer 2020 Mar;20(3):158-173. Epub 2020 Jan 6 doi: 10.1038/s41568-019-0230-9. PMID: 31907378

Recent systematic reviews

Wu A, Liu X, Fruhstorfer C, Jiang X
Int J Mol Sci 2024 Mar 14;25(6) doi: 10.3390/ijms25063307. PMID: 38542279Free PMC Article
Lipton JH
Clin Drug Investig 2024 Feb;44(2):91-108. Epub 2024 Jan 5 doi: 10.1007/s40261-023-01329-9. PMID: 38182963
Zhang Y, Chen W, Pathak A, Yang Y, Yang D, Zhai Y
J Comp Eff Res 2022 Jun;11(8):621-637. Epub 2022 Apr 12 doi: 10.2217/cer-2022-0032. PMID: 35411807
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
Ercaliskan A, Eskazan AE
Cancer 2018 Oct 1;124(19):3806-3818. Epub 2018 Apr 25 doi: 10.1002/cncr.31408. PMID: 29694669

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