The WHO 2016 diagnostic criteria for Acute Myeloid leukemia with myelodysplasia related changes (AML-MRC) produce a very heterogeneous entity: A retrospective analysis of the FAB subtype RAEB-T. (January 2022)

• Record Type: Journal Article
• Authors: Kaivers, J.; Peters, J.; Rautenberg, C.; Schroeder, T.; Kobbe, G.; Hildebrandt, B.; Haas, R.; Germing, U.; Bennett, J.M.
• Is Part Of: Leukemia research. Volume 112(2022)
• Publication Date: 2022-01
• Subjects: Acute Myeloid Leukemia -- Myelodysplasia-related changes -- Myelodysplastic syndromes -- RAEB-T; Leukemia -- Periodicals; Leukemia -- Periodicals; Leucémie -- Périodiques; Leukemia; Periodicals; Electronic journals; Electronic journals; 616.9941905
• DOI: 10.1016/j.leukres.2021.106757
• Languages: English
• ISSNs: 0145-2126

Abstract

Highlights: MDS-specific cytogenetic changes are the most frequent criterion for the diagnosis of AML-MRC (54.4 %), of those 74.4 % have a complex karyotype. Multilineage dysplasia in AML-MRC is the least frequent criterion and can only be verified in 30.4 % of all cases. AML-MRC has the highest rates of complex karyotypes compared to other AML subtypes and high-risk MDS. AML-MRC (together with tAML) has the worst prognosis with a median OS of 9, 1 months only. Abstract: We studied 79 patients with AML-MRC or RAEB-T, who were later reclassified according to the WHO classification. Marrow slides were examined cytomorphologically with regard to dysplasia. Patients were followed up until March 2020. Thirty-one patients underwent allogeneic stem cell transplantation (median survival (ms) 16 months), 14 were treated with induction chemotherapy (ms 8.4 months), 18 received hypomethylating agents (ms 9.2 months), 16 received low dose chemotherapy or best supportive care (ms 2.4 months). Only 30.4 % fulfilled the morphologic WHO criteria. 46.8 % were classified as AML-MRC by an antecedent MDS, 54.4 % of the pts were classified by MDS-related chromosomal abnormalities. 5 % did not fulfill any of the criteria and were entered based on 20–29 % medullary blasts. There was no difference in ms between pts presenting with > 50 % dysplasia as compared to pts with dysplasia between 10 % and 50 % (ms 9.1 vs 9.9 months, p = n.s.) or for pts with antecedent MDS (ms 9.1 vs 8.9 months, p = n.s.). Myelodysplasia-related cytogenetic abnormalities were associated with a worse outcome (ms 8.1 vs 13.5 months, p = 0.026). AML-MRC in its current definition is a heterogenous entity. Dysplasia of ≥ 50 % in ≥ two lineages is not helpful for diagnostics and prognostication and therefore should be deleted in future classifications. We recommend utilizing the WHO guidelines for defining dysplasia (10 % or greater in ≥ 1 of the three myeloid cell lines) assisting in establishing the diagnosis of MDS.