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Acute myeloid leukemia, M6 type

MedGen UID:
7316
•Concept ID:
C0023440
•
Neoplastic Process
Synonyms: Acute erythroid leukemia; Acute erythroleukemia; Acute erythroleukemia - M6a subtype; Acute erythroleukemia - M6b subtype; Acute myeloid leukemia FAB-M6; Di Guglielmo's syndrome; Erythroleukemia, FAB M6
SNOMED CT: Acute erythroid leukemia (14317002); Acute myeloid leukemia, M6 type (14317002); Di Guglielmo's disease (93451002); Erythroleukemia, FAB M6 (93451002); Erythroleukemia (14317002); Erythremic myelosis (14317002); AML (acute myeloid leukemia), M6 type (14317002); Erythremic myelosis (93451002); M6 - Acute erythroleukemia (93451002); Erythroleukemia (93451002)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Related gene: ERBB3
 
Monarch Initiative: MONDO:0017858
Orphanet: ORPHA318

Definition

A rare unclassified acute myeloid leukemia characterized by a proliferation of immature cells exclusively of the erythroid lineage without a significant myeloblastic component. Microscopically, the cells may be undifferentiated or proerythroblastic in appearance. Patients may present with pancytopenia with fatigue, infections, and mucocutaneous bleedings, as well as weight loss, fever, and night sweats. Prognosis is poor. [from ORDO]

Professional guidelines

PubMed

Pure (acute) erythroid leukemia: morphology, immunophenotype, cytogenetics, mutations, treatment details, and survival data among 41 Mayo Clinic cases.
Reichard KK, Tefferi A, Abdelmagid M, Orazi A, Alexandres C, Haack J, Greipp PT
Blood Cancer J 2022 Nov 2;12(11):147. doi: 10.1038/s41408-022-00746-x. PMID: 36323674Free PMC Article
Erythroleukemia-historical perspectives and recent advances in diagnosis and management.
Boddu P, Benton CB, Wang W, Borthakur G, Khoury JD, Pemmaraju N
Blood Rev 2018 Mar;32(2):96-105. Epub 2017 Sep 18 doi: 10.1016/j.blre.2017.09.002. PMID: 28965757Free PMC Article
Acute erythroleukemia: diagnosis and management.
Santos FP, Bueso-Ramos CE, Ravandi F
Expert Rev Hematol 2010 Dec;3(6):705-18. doi: 10.1586/ehm.10.62. PMID: 21091147

Recent clinical studies

Etiology

m(6)A-driven SF3B1 translation control steers splicing to direct genome integrity and leukemogenesis.
Cieśla M, Ngoc PCT, Muthukumar S, Todisco G, Madej M, Fritz H, Dimitriou M, Incarnato D, Hellström-Lindberg E, Bellodi C
Mol Cell 2023 Apr 6;83(7):1165-1179.e11. Epub 2023 Mar 20 doi: 10.1016/j.molcel.2023.02.024. PMID: 36944332
Erythroleukemia: an Update.
Weinberg OK, Arber DA
Curr Oncol Rep 2021 Apr 20;23(6):69. doi: 10.1007/s11912-021-01060-8. PMID: 33876292
Revisiting erythroleukemia.
Arber DA
Curr Opin Hematol 2017 Mar;24(2):146-151. doi: 10.1097/MOH.0000000000000314. PMID: 27875373
Gene methylation in gastric cancer.
Qu Y, Dang S, Hou P
Clin Chim Acta 2013 Sep 23;424:53-65. Epub 2013 May 10 doi: 10.1016/j.cca.2013.05.002. PMID: 23669186
Proposals for the classification of the acute leukaemias. French-American-British (FAB) co-operative group.
Bennett JM, Catovsky D, Daniel MT, Flandrin G, Galton DA, Gralnick HR, Sultan C
Br J Haematol 1976 Aug;33(4):451-8. doi: 10.1111/j.1365-2141.1976.tb03563.x. PMID: 188440

Diagnosis

A History and Current Understanding of Acute Erythroid Leukemia.
Alexander C
Clin Lymphoma Myeloma Leuk 2023 Aug;23(8):583-588. Epub 2023 Apr 28 doi: 10.1016/j.clml.2023.04.011. PMID: 37246017
Erythroleukemia: an Update.
Weinberg OK, Arber DA
Curr Oncol Rep 2021 Apr 20;23(6):69. doi: 10.1007/s11912-021-01060-8. PMID: 33876292
Pure erythroid leukemia.
Wang W, Wang SA, Medeiros LJ, Khoury JD
Am J Hematol 2017 Mar;92(3):292-296. Epub 2017 Feb 3 doi: 10.1002/ajh.24626. PMID: 28006859
Revisiting erythroleukemia.
Arber DA
Curr Opin Hematol 2017 Mar;24(2):146-151. doi: 10.1097/MOH.0000000000000314. PMID: 27875373
Proposals for the classification of the acute leukaemias. French-American-British (FAB) co-operative group.
Bennett JM, Catovsky D, Daniel MT, Flandrin G, Galton DA, Gralnick HR, Sultan C
Br J Haematol 1976 Aug;33(4):451-8. doi: 10.1111/j.1365-2141.1976.tb03563.x. PMID: 188440

Therapy

m(6)Acancer-Net: Identification of m(6)A-mediated cancer driver genes from gene-site heterogeneous network.
Ma Q, Zhang SW, Zhang SY
Methods 2022 Jul;203:125-138. Epub 2022 Apr 15 doi: 10.1016/j.ymeth.2022.04.002. PMID: 35436514
Di-O-lauroyl-decitabine-lipid nanocapsules: toward extending decitabine activity.
Briot T, Roger E, Bou Haidar N, Bejaud J, Lautram N, Guillet C, Thépot S, Legeay S, Lagarce F
Int J Nanomedicine 2019;14:2091-2102. Epub 2019 Mar 26 doi: 10.2147/IJN.S190482. PMID: 30988610Free PMC Article
Development of a curative treatment within the AML-BFM studies.
Creutzig U, Zimmermann M, Dworzak MN, Ritter J, Schellong G, Reinhardt D
Klin Padiatr 2013 May;225 Suppl 1:S79-86. Epub 2013 May 22 doi: 10.1055/s-0033-1337968. PMID: 23700063
Established leukemia cell lines: their role in the understanding and control of leukemia proliferation.
Hozumi M
Crit Rev Oncol Hematol 1985;3(3):235-77. doi: 10.1016/s1040-8428(85)80028-7. PMID: 3899390
Hodgkin's disease and acute leukemia. Report of eight cases and review of the literature.
Rosner F, Grünwald H
Am J Med 1975 Mar;58(3):339-53. doi: 10.1016/0002-9343(75)90600-2. PMID: 1090158

Prognosis

m(6)A-driven SF3B1 translation control steers splicing to direct genome integrity and leukemogenesis.
Cieśla M, Ngoc PCT, Muthukumar S, Todisco G, Madej M, Fritz H, Dimitriou M, Incarnato D, Hellström-Lindberg E, Bellodi C
Mol Cell 2023 Apr 6;83(7):1165-1179.e11. Epub 2023 Mar 20 doi: 10.1016/j.molcel.2023.02.024. PMID: 36944332
Erythroleukemia: an Update.
Weinberg OK, Arber DA
Curr Oncol Rep 2021 Apr 20;23(6):69. doi: 10.1007/s11912-021-01060-8. PMID: 33876292
To bi or not to bi: Acute erythroid leukemias and hematopoietic lineage choice.
Di Genua C, Nerlov C
Exp Hematol 2021 May;97:6-13. Epub 2021 Feb 16 doi: 10.1016/j.exphem.2021.02.006. PMID: 33600869
Chemotherapeutics for Acute Erythroid Leukemia: Research, Present and Future.
Liao Z, Li J, Wu J, Liu J, Sun S
Curr Mol Med 2021;21(10):819-831. doi: 10.2174/1566524021666210121142316. PMID: 33475071
Revisiting erythroleukemia.
Arber DA
Curr Opin Hematol 2017 Mar;24(2):146-151. doi: 10.1097/MOH.0000000000000314. PMID: 27875373

Clinical prediction guides

A reevaluation of erythroid predominance in Acute Myeloid Leukemia using the updated WHO 2016 Criteria.
Margolskee E, Mikita G, Rea B, Bagg A, Zuo Z, Sun Y, Goswami M, Wang SA, Oak J, Arber DA, Allen MB, George TI, Rogers HJ, Hsi E, Hasserjian RP, Orazi A
Mod Pathol 2018 Jun;31(6):873-880. Epub 2018 Feb 5 doi: 10.1038/s41379-018-0001-2. PMID: 29403082
Acute megakaryoblastic leukemia, unlike acute erythroid leukemia, predicts an unfavorable outcome after allogeneic HSCT.
Ishiyama K, Yamaguchi T, Eto T, Ohashi K, Uchida N, Kanamori H, Fukuda T, Miyamura K, Inoue Y, Taguchi J, Mori T, Iwato K, Morishima Y, Nagamura-Inoue T, Atsuta Y, Sakamaki H, Takami A
Leuk Res 2016 Aug;47:47-53. Epub 2016 May 12 doi: 10.1016/j.leukres.2016.04.017. PMID: 27244257
Incidence and geographic distribution of adult acute leukemia in the state of Georgia.
El Rassi F, Ward KC, Flowers CR, Heffner LT, Waller EK, Winton EF, Vaughn J, Hill BG, Langston A, Nooka A, Arellano M, Khoury HJ
South Med J 2014 Aug;107(8):497-500. doi: 10.14423/SMJ.0000000000000146. PMID: 25084186
Acute erythroid neoplastic proliferations. A biological study based on 62 patients.
Domingo-Claros A, Larriba I, Rozman M, Irriguible D, Vallespí T, Aventin A, Ayats R, Millá F, Solé F, Florensa L, Gallart M, Tuset E, Lopez C, Woessner S
Haematologica 2002 Feb;87(2):148-53. PMID: 11836165
Morphologic characteristics of erythroleukemia (acute myeloid leukemia; FAB-M6): a CALGB study.
Davey FR, Abraham N Jr, Brunetto VL, MacCallum JM, Nelson DA, Ball ED, Griffin JD, Baer MR, Wurster-Hill D, Mayer RJ
Am J Hematol 1995 May;49(1):29-38. doi: 10.1002/ajh.2830490106. PMID: 7741135

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