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Adult Acute Promyelocytic Leukemia with PML-RARA

MedGen UID:
83535
•Concept ID:
C0279625
•
Neoplastic Process
Synonyms: Adult Acute M3 Leukemia; Adult Acute Promyelocytic Leukemia; Adult Acute Promyelocytic Leukemia (M3); Adult Acute Promyelocytic Leukemia with PML::RARA; Adult Acute Promyelocytic Leukemia with t(15;17)(q22;q12)(PML/RARa); Adult Acute Promyelocytic Leukemia with t(15;17)(q22;q12); PML-RARA; Adult Acute Promyelocytic Leukemia with t(15;17)(q22;q12); PML/RARA; Adult APL; M3 Adult Acute Leukemia; M3 Adult Acute Promyelocytic Leukemia

Definition

An acute promyelocytic leukemia with PML-RARA fusion occurring in adults. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdult Acute Promyelocytic Leukemia with PML-RARA

Professional guidelines

PubMed

Acute Promyelocytic Leukemia: Update on Risk Stratification and Treatment Practices.
Salhotra A, Mei M
Cancer Treat Res 2021;181:45-55. doi: 10.1007/978-3-030-78311-2_3. PMID: 34626354
NCCN Guidelines Insights: Acute Myeloid Leukemia, Version 2.2021.
Pollyea DA, Bixby D, Perl A, Bhatt VR, Altman JK, Appelbaum FR, de Lima M, Fathi AT, Foran JM, Gojo I, Hall AC, Jacoby M, Lancet J, Mannis G, Marcucci G, Martin MG, Mims A, Neff J, Nejati R, Olin R, Percival ME, Prebet T, Przespolewski A, Rao D, Ravandi-Kashani F, Shami PJ, Stone RM, Strickland SA, Sweet K, Vachhani P, Wieduwilt M, Gregory KM, Ogba N, Tallman MS
J Natl Compr Canc Netw 2021 Jan 6;19(1):16-27. doi: 10.6004/jnccn.2021.0002. PMID: 33406488
Management of acute promyelocytic leukemia: updated recommendations from an expert panel of the European LeukemiaNet.
Sanz MA, Fenaux P, Tallman MS, Estey EH, Löwenberg B, Naoe T, Lengfelder E, Döhner H, Burnett AK, Chen SJ, Mathews V, Iland H, Rego E, Kantarjian H, Adès L, Avvisati G, Montesinos P, Platzbecker U, Ravandi F, Russell NH, Lo-Coco F
Blood 2019 Apr 11;133(15):1630-1643. Epub 2019 Feb 25 doi: 10.1182/blood-2019-01-894980. PMID: 30803991Free PMC Article

Recent clinical studies

Etiology

Assessment of Arsenic Trioxide and All-trans Retinoic Acid for the Treatment of Pediatric Acute Promyelocytic Leukemia: A Report From the Children's Oncology Group AAML1331 Trial.
Kutny MA, Alonzo TA, Abla O, Rajpurkar M, Gerbing RB, Wang YC, Hirsch BA, Raimondi S, Kahwash S, Hardy KK, Hardy S, Meshinchi S, Gamis AS, Kolb EA, Feusner JH, Gregory J Jr
JAMA Oncol 2022 Jan 1;8(1):79-87. doi: 10.1001/jamaoncol.2021.5206. PMID: 34762093Free PMC Article
Acute Promyelocytic Leukemia: Update on Risk Stratification and Treatment Practices.
Salhotra A, Mei M
Cancer Treat Res 2021;181:45-55. doi: 10.1007/978-3-030-78311-2_3. PMID: 34626354
Management of acute promyelocytic leukemia: updated recommendations from an expert panel of the European LeukemiaNet.
Sanz MA, Fenaux P, Tallman MS, Estey EH, Löwenberg B, Naoe T, Lengfelder E, Döhner H, Burnett AK, Chen SJ, Mathews V, Iland H, Rego E, Kantarjian H, Adès L, Avvisati G, Montesinos P, Platzbecker U, Ravandi F, Russell NH, Lo-Coco F
Blood 2019 Apr 11;133(15):1630-1643. Epub 2019 Feb 25 doi: 10.1182/blood-2019-01-894980. PMID: 30803991Free PMC Article
Arsenic trioxide and all-trans retinoic acid treatment for acute promyelocytic leukaemia in all risk groups (AML17): results of a randomised, controlled, phase 3 trial.
Burnett AK, Russell NH, Hills RK, Bowen D, Kell J, Knapper S, Morgan YG, Lok J, Grech A, Jones G, Khwaja A, Friis L, McMullin MF, Hunter A, Clark RE, Grimwade D; UK National Cancer Research Institute Acute Myeloid Leukaemia Working Group
Lancet Oncol 2015 Oct;16(13):1295-305. Epub 2015 Sep 14 doi: 10.1016/S1470-2045(15)00193-X. PMID: 26384238
Adult acute leukemia.
Cornell RF, Palmer J
Dis Mon 2012 Apr;58(4):219-38. doi: 10.1016/j.disamonth.2012.01.011. PMID: 22449370

Diagnosis

Acute Promyelocytic Leukemia: Update on Risk Stratification and Treatment Practices.
Salhotra A, Mei M
Cancer Treat Res 2021;181:45-55. doi: 10.1007/978-3-030-78311-2_3. PMID: 34626354
Management of acute promyelocytic leukemia: updated recommendations from an expert panel of the European LeukemiaNet.
Sanz MA, Fenaux P, Tallman MS, Estey EH, Löwenberg B, Naoe T, Lengfelder E, Döhner H, Burnett AK, Chen SJ, Mathews V, Iland H, Rego E, Kantarjian H, Adès L, Avvisati G, Montesinos P, Platzbecker U, Ravandi F, Russell NH, Lo-Coco F
Blood 2019 Apr 11;133(15):1630-1643. Epub 2019 Feb 25 doi: 10.1182/blood-2019-01-894980. PMID: 30803991Free PMC Article
Treatment of Acute Promyelocytic Leukemia in Adults.
Osman AEG, Anderson J, Churpek JE, Christ TN, Curran E, Godley LA, Liu H, Thirman MJ, Odenike T, Stock W, Larson RA
J Oncol Pract 2018 Nov;14(11):649-657. doi: 10.1200/JOP.18.00328. PMID: 30423270
Current standard treatment of adult acute promyelocytic leukaemia.
Lo-Coco F, Cicconi L, Breccia M
Br J Haematol 2016 Mar;172(6):841-54. Epub 2015 Dec 21 doi: 10.1111/bjh.13890. PMID: 26687281
Arsenic trioxide and all-trans retinoic acid treatment for acute promyelocytic leukaemia in all risk groups (AML17): results of a randomised, controlled, phase 3 trial.
Burnett AK, Russell NH, Hills RK, Bowen D, Kell J, Knapper S, Morgan YG, Lok J, Grech A, Jones G, Khwaja A, Friis L, McMullin MF, Hunter A, Clark RE, Grimwade D; UK National Cancer Research Institute Acute Myeloid Leukaemia Working Group
Lancet Oncol 2015 Oct;16(13):1295-305. Epub 2015 Sep 14 doi: 10.1016/S1470-2045(15)00193-X. PMID: 26384238

Therapy

Acute Promyelocytic Leukemia.
Winkler C
N Engl J Med 2024 May 2;390(17):e42. Epub 2024 Apr 27 doi: 10.1056/NEJMicm2314162. PMID: 38683728
Management of acute promyelocytic leukemia: updated recommendations from an expert panel of the European LeukemiaNet.
Sanz MA, Fenaux P, Tallman MS, Estey EH, Löwenberg B, Naoe T, Lengfelder E, Döhner H, Burnett AK, Chen SJ, Mathews V, Iland H, Rego E, Kantarjian H, Adès L, Avvisati G, Montesinos P, Platzbecker U, Ravandi F, Russell NH, Lo-Coco F
Blood 2019 Apr 11;133(15):1630-1643. Epub 2019 Feb 25 doi: 10.1182/blood-2019-01-894980. PMID: 30803991Free PMC Article
Treatment of Acute Promyelocytic Leukemia in Adults.
Osman AEG, Anderson J, Churpek JE, Christ TN, Curran E, Godley LA, Liu H, Thirman MJ, Odenike T, Stock W, Larson RA
J Oncol Pract 2018 Nov;14(11):649-657. doi: 10.1200/JOP.18.00328. PMID: 30423270
Current standard treatment of adult acute promyelocytic leukaemia.
Lo-Coco F, Cicconi L, Breccia M
Br J Haematol 2016 Mar;172(6):841-54. Epub 2015 Dec 21 doi: 10.1111/bjh.13890. PMID: 26687281
Arsenic trioxide and all-trans retinoic acid treatment for acute promyelocytic leukaemia in all risk groups (AML17): results of a randomised, controlled, phase 3 trial.
Burnett AK, Russell NH, Hills RK, Bowen D, Kell J, Knapper S, Morgan YG, Lok J, Grech A, Jones G, Khwaja A, Friis L, McMullin MF, Hunter A, Clark RE, Grimwade D; UK National Cancer Research Institute Acute Myeloid Leukaemia Working Group
Lancet Oncol 2015 Oct;16(13):1295-305. Epub 2015 Sep 14 doi: 10.1016/S1470-2045(15)00193-X. PMID: 26384238

Prognosis

Predictive factors of fatal bleeding in acute promyelocytic leukemia.
Mantha S, Tallman MS, Devlin SM, Soff GA
Thromb Res 2018 Apr;164 Suppl 1:S98-S102. doi: 10.1016/j.thromres.2018.01.038. PMID: 29703492
Acute promyelocytic leukemia: preventing early complications and late toxicities.
Abedin S, Altman JK
Hematology Am Soc Hematol Educ Program 2016 Dec 2;2016(1):10-15. doi: 10.1182/asheducation-2016.1.10. PMID: 27913456Free PMC Article
Arsenic trioxide and all-trans retinoic acid treatment for acute promyelocytic leukaemia in all risk groups (AML17): results of a randomised, controlled, phase 3 trial.
Burnett AK, Russell NH, Hills RK, Bowen D, Kell J, Knapper S, Morgan YG, Lok J, Grech A, Jones G, Khwaja A, Friis L, McMullin MF, Hunter A, Clark RE, Grimwade D; UK National Cancer Research Institute Acute Myeloid Leukaemia Working Group
Lancet Oncol 2015 Oct;16(13):1295-305. Epub 2015 Sep 14 doi: 10.1016/S1470-2045(15)00193-X. PMID: 26384238
Acute promyelocytic leukemia in children and adolescents.
Stein EM, Tallman MS
Acta Haematol 2014;132(3-4):307-12. Epub 2014 Sep 10 doi: 10.1159/000365117. PMID: 25228556
Management of elderly patients with acute promyelocytic leukemia: progress and problems.
Lengfelder E, Hofmann WK, Nolte F
Ann Hematol 2013 Sep;92(9):1181-8. Epub 2013 May 22 doi: 10.1007/s00277-013-1788-z. PMID: 23694997Free PMC Article

Clinical prediction guides

Long-term outcome of children with acute promyelocytic leukemia: a randomized study of oral versus intravenous arsenic by SCCLG-APL group.
Huang DP, Yang LC, Chen YQ, Wan WQ, Zhou DH, Mai HR, Li WL, Yang LH, Lan HK, Chen HQ, Guo BY, Zhen ZJ, Liu RY, Chen GH, Feng XQ, Liang C, Wang LN, Li Y, Luo JS, Fan Z, Luo XQ, Li B, Tang YL, Zhang XL, Huang LB
Blood Cancer J 2023 Dec 5;13(1):178. doi: 10.1038/s41408-023-00949-w. PMID: 38052803Free PMC Article
Mutational profile of ZBTB16-RARA-positive acute myeloid leukemia.
Fabiani E, Cicconi L, Nardozza AM, Cristiano A, Rossi M, Ottone T, Falconi G, Divona M, Testi AM, Annibali O, Castelli R, Lazarevic V, Rego E, Montesinos P, Esteve J, Venditti A, Della Porta M, Arcese W, Lo-Coco F, Voso MT
Cancer Med 2021 Jun;10(12):3839-3847. Epub 2021 May 27 doi: 10.1002/cam4.3904. PMID: 34042280Free PMC Article
Predictive factors of fatal bleeding in acute promyelocytic leukemia.
Mantha S, Tallman MS, Devlin SM, Soff GA
Thromb Res 2018 Apr;164 Suppl 1:S98-S102. doi: 10.1016/j.thromres.2018.01.038. PMID: 29703492
Acute promyelocytic leukemia-associated thrombosis.
Chang H, Kuo MC, Shih LY, Wu JH, Lin TL, Dunn P, Tang TC, Hung YS, Wang PN
Acta Haematol 2013;130(1):1-6. Epub 2013 Jan 23 doi: 10.1159/000345833. PMID: 23343825
Adult acute leukemia.
Cripe LD
Curr Probl Cancer 1997 Jan-Feb;21(1):1-64. doi: 10.1016/s0147-0272(97)80006-2. PMID: 9058027

Recent systematic reviews

Health-related quality of life in patients with acute promyelocytic leukemia: a systematic literature review.
Efficace F, Cannella L, Breccia M, Olivieri J, Platzbecker U, Vignetti M
Expert Rev Hematol 2021 Jul;14(7):645-654. Epub 2021 Jul 26 doi: 10.1080/17474086.2021.1943352. PMID: 34125642
Acute promyelocytic leukemia during pregnancy: a systematic analysis of outcome.
Verma V, Giri S, Manandhar S, Pathak R, Bhatt VR
Leuk Lymphoma 2016;57(3):616-22. Epub 2015 Jul 28 doi: 10.3109/10428194.2015.1065977. PMID: 26110880
Therapy-related acute promyelocytic leukemia: a systematic review.
Rashidi A, Fisher SI
Med Oncol 2013;30(3):625. Epub 2013 Jun 15 doi: 10.1007/s12032-013-0625-5. PMID: 23771799
Lack of clarity in the definition of treatment-related mortality: pediatric acute leukemia and adult acute promyelocytic leukemia as examples.
Ethier MC, Blanco E, Lehrnbecher T, Sung L
Blood 2011 Nov 10;118(19):5080-3. Epub 2011 Sep 21 doi: 10.1182/blood-2011-07-363333. PMID: 21937689
Prognostic significance of FLT3 internal tandem duplication and tyrosine kinase domain mutations in acute promyelocytic leukemia: a systematic review.
Beitinjaneh A, Jang S, Roukoz H, Majhail NS
Leuk Res 2010 Jul;34(7):831-6. Epub 2010 Jan 21 doi: 10.1016/j.leukres.2010.01.001. PMID: 20096459

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