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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

Acute erythroid leukemia with TP53 mutation and BCR/ABL1: challenges in classification and management.
Marra JD, Frioni F, Minnella G, Rossi M, Malara T, Bellesi S, Maiolo E, Orteschi D, Galli E, Bacigalupo A, Pagano L, Sica S, Zini G, Chiusolo P
Ann Hematol 2024 Mar;103(3):1013-1014. Epub 2023 Nov 29 doi: 10.1007/s00277-023-05561-3. PMID: 38017337
Diagnostic criteria for acute erythroleukemia.
Selby DM, Valdez R, Schnitzer B, Ross CW, Finn WG
Blood 2003 Apr 1;101(7):2895-6. doi: 10.1182/blood-2002-11-3459. PMID: 12642345
Uncommon subtypes of acute nonlymphocytic leukemia: clinical features and management of FAB M5, M6 and M7.
Peterson BA, Levine EG
Semin Oncol 1987 Dec;14(4):425-34. PMID: 3321447

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

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
The myeloproliferative disorders.
Gilbert HS, Dameshek W
Dis Mon 1970 Oct:1-52. doi: 10.1016/s0011-5029(70)80012-8. PMID: 4921953

Therapy

Subacute anthracycline cardiotoxicity.
Hengel CL, Russell PA, Gould PA, Kaye DM
Heart Lung Circ 2006 Feb;15(1):59-61. Epub 2005 Nov 11 doi: 10.1016/j.hlc.2005.06.003. PMID: 16473794
Uncommon subtypes of acute nonlymphocytic leukemia: clinical features and management of FAB M5, M6 and M7.
Peterson BA, Levine EG
Semin Oncol 1987 Dec;14(4):425-34. PMID: 3321447
The deoxyuridine suppression test.
Metz J
Crit Rev Clin Lab Sci 1984;20(3):205-41. doi: 10.3109/10408368409165775. PMID: 6426860
Chemical leukemogenesis in man.
Forni A, Vigliani EC
Ser Haematol 1974;7(2):211-23. PMID: 4610755
Paraplegia following intrathecal chemotherapy.
Saiki JH, Thompson S, Smith F, Atkinson R
Cancer 1972 Feb;29(2):370-4. doi: 10.1002/1097-0142(197202)29:2<370::aid-cncr2820290217>3.0.co;2-z. PMID: 4552135

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
Revisiting erythroleukemia.
Arber DA
Curr Opin Hematol 2017 Mar;24(2):146-151. doi: 10.1097/MOH.0000000000000314. PMID: 27875373
Novel germ line DDX41 mutations define families with a lower age of MDS/AML onset and lymphoid malignancies.
Lewinsohn M, Brown AL, Weinel LM, Phung C, Rafidi G, Lee MK, Schreiber AW, Feng J, Babic M, Chong CE, Lee Y, Yong A, Suthers GK, Poplawski N, Altree M, Phillips K, Jaensch L, Fine M, D'Andrea RJ, Lewis ID, Medeiros BC, Pollyea DA, King MC, Walsh T, Keel S, Shimamura A, Godley LA, Hahn CN, Churpek JE, Scott HS
Blood 2016 Feb 25;127(8):1017-23. Epub 2015 Dec 28 doi: 10.1182/blood-2015-10-676098. PMID: 26712909Free PMC Article
Acute erythroid leukemia.
Zuo Z, Polski JM, Kasyan A, Medeiros LJ
Arch Pathol Lab Med 2010 Sep;134(9):1261-70. doi: 10.5858/2009-0350-RA.1. PMID: 20807044

Clinical prediction guides

A novel, somatic, transforming mutation in the extracellular domain of Epidermal Growth Factor Receptor identified in myeloproliferative neoplasm.
Casolari DA, Nguyen T, Butcher CM, Iarossi DG, Hahn CN, Bray SC, Neufing P, Parker WT, Feng J, Maung KZY, Wee A, Vidovic L, Kok CH, Bardy PG, Branford S, Lewis ID, Lane SW, Scott HS, Ross DM, D'Andrea RJ
Sci Rep 2017 May 26;7(1):2467. doi: 10.1038/s41598-017-02655-7. PMID: 28550306Free PMC Article
Interleukin-6 directly impairs the erythroid development of human TF-1 erythroleukemic cells.
McCranor BJ, Kim MJ, Cruz NM, Xue QL, Berger AE, Walston JD, Civin CI, Roy CN
Blood Cells Mol Dis 2014 Feb-Mar;52(2-3):126-33. Epub 2013 Oct 9 doi: 10.1016/j.bcmd.2013.09.004. PMID: 24119518Free PMC Article
Translational regulation of human p53 gene expression.
Fu L, Minden MD, Benchimol S
EMBO J 1996 Aug 15;15(16):4392-401. PMID: 8861966Free PMC Article
Human large granular lymphocytes and natural killing ultrastructural studies of strontium-induced degranulation.
Neighbour PA, Huberman HS, Kress Y
Eur J Immunol 1982 Jul;12(7):588-95. doi: 10.1002/eji.1830120711. PMID: 6956515
Dyserythropoiesis and annulate lamellae.
Verwilghen RL, Broeckaert-Van Orshoven A, Heynen MJ
Br J Haematol 1975 Jul;30(3):307-10. doi: 10.1111/j.1365-2141.1975.tb00546.x. PMID: 1059475

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