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The non-erythroid myeloblast count rule in myelodysplastic syndromes: fruitful or futile?
Margot F. van Spronsen, Theresia M. Westers, Birgit I. Lissenberg-Witte, Mariëlle Wondergem, Gert J. Ossenkoppele, Arjan A. van de Loosdrecht
Haematologica December 2019 104: e547-e550; doi:10.3324/haematol.2018.212563
Margot F. van Spronsen
Department of Hematology, Amsterdam UMC, location Vrije Universiteit Amsterdam, Cancer Center Amsterdam
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Theresia M. Westers
Department of Hematology, Amsterdam UMC, location Vrije Universiteit Amsterdam, Cancer Center Amsterdam
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Birgit I. Lissenberg-Witte
Department of Epidemiology and Biostatistics, Amsterdam UMC, location Vrije Universiteit Amsterdam, Amsterdam, the Netherlands
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Mariëlle Wondergem
Department of Hematology, Amsterdam UMC, location Vrije Universiteit Amsterdam, Cancer Center Amsterdam
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Gert J. Ossenkoppele
Department of Hematology, Amsterdam UMC, location Vrije Universiteit Amsterdam, Cancer Center Amsterdam
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Arjan A. van de Loosdrecht
Department of Hematology, Amsterdam UMC, location Vrije Universiteit Amsterdam, Cancer Center Amsterdam
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  • For correspondence: a.vandeloosdrecht@vumc.nl

Author Affiliations

  1. Margot F. van Spronsen1,
  2. Theresia M. Westers1,
  3. Birgit I. Lissenberg-Witte2,
  4. Mariëlle Wondergem1,
  5. Gert J. Ossenkoppele1 and
  6. Arjan A. van de Loosdrecht1⇑
  1. 1Department of Hematology, Amsterdam UMC, location Vrije Universiteit Amsterdam, Cancer Center Amsterdam
  2. 2Department of Epidemiology and Biostatistics, Amsterdam UMC, location Vrije Universiteit Amsterdam, Amsterdam, the Netherlands
  1. Correspondence: ARJAN A. VAN DE LOOSDRECHT: a.vandeloosdrecht{at}vumc.nl
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Data supplements

  • van Spronsen et al. Supplementary Appendix

    van Spronsen et al. Supplementary appendix provided by the authors.

    Files in this Data Supplement:

    • Adobe PDF - 2018.212563.VAN_SPRONSEN_SUPPL.pdf
  • Disclosures and Contributions

    Files in this Data Supplement:

    • Adobe PDF - 2018_212563-Disclosures_and_Contributions.pdf

ARTICLE FIGURES & DATA

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    Figure 1.

    Study on the value of the non-erythroid blast count (NEBC) rule. All Kaplan-Meier estimates illustrate the overall survival (OS) and leukemia-free survival (LFS) times in months. (A) Marrow erythroid cell- and myeloblast percentages from total bone marrow cells distributed by diagnosis according to the World Health Organization (WHO) 2008 and 2016 criteria. (B) MDS-E patients myelodysplastic syndromes with erythroid predominance show an improved clinical outcome as compared to MDS-NE patients in spite of the used WHO criteria. (C) WHO 2016 and MDS-NE patients show a comparable clinical outcome when stratifying by marrow myeloblast percentages, except for MDS patients with ≥11% myeloblasts in spite of the small sample size. (D) The WHO 2008 and 2016 classify less MDS-E than MDS-NE patients as refractory anemia with excess blasts type 2 (RAEB-2) and excess blasts type 2 (EB-2). (E) Impact of the use of the NEBC rule within the revised International Prognostic Scoring System (IPSS-R) and WHO 2016 classification. The number refers to the absolute amount of patients classified according to the IPSS-R and WHO 2016 classification without use of the NEBC rule (in green) and with use of the NEBC rule (in orange). (F) Clinical outcome of MDS-E patients initially classified as IPSS-R very low- or low-, IPSS-R intermediate-, IPSS-R high- or very high- and WHO low-risk who are upgraded to a higher risk category by the NEBC rule in comparison with MDS-E and MDS-NE patients who remained classified into initial categories. WHO low-risk is defined as <2% and <5% peripheral blood- and bone marrow myeloblast percentages, respectively. del(5q): MDS with isolated del (5q); RCUD: refractory cytopenia with unilineage dysplasia; RARS: refractory anemia with ring sideroblasts; RCMD: refractory cytopenia with multilineage dysplasia; MDS-U: MDS unclassifiable; RAEB-1: refractory anemia with excess blasts type 1; SLD: single lineage dysplasia with ring sideroblasts; MLD: multilineage dysplasia; RS-SLD: single lineage dysplasia with ring sideroblasts; RS-MLD: multilineage dysplasia with ring sideroblasts; EB-1: excess blasts type 1; EB-2: excess blasts type 2.

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    Figure 2.

    Study on the nature of erythroid predominance. (A) The absence or presence of erythroid predominance defined as ≥ 50% marrow erythroid cells at median follow-up time of 35 (1-119) and 16 (1-119) months in World Health Organization (WHO) 2016 myelodysplastic syndromes (MDS) with erythroid predominance (MDS-E) and MDS with non-erythroid predominance (MDS-NE) patients, respectively. Half of the MDS-E patients have either normalization or persistence of erythroid predominance. Most MDS-NE patients maintain a non-erythroid predominant bone marrow. (B) The prognostic value of distinct marrow erythroid cell percentages. MDS patients with less than 15% or at least 80% marrow erythroid cells have the poorest clinical outcome. (C) K-means clustering based on bone marrow and peripheral blood cell counts was applied to identify patient clusters. Note that K-means clustering was based on all these features except for the percentage of peripheral blood myeloblasts. (D) Clinical outcome of WHO 2016 MDS patients stratified by K-means clustering. Cluster 2 and 3, the aggressive erythroid predominance and myeloid predominance subtype, respectively, show the poorest clinical outcome. All Kaplan-Meier estimates illustrate the overall survival (OS) and leukemia-free survival (LFS) times in months.

Tables

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  • Table 1.

    Observations on the use of the non-erythroid blast count rule and the clinicopathological characterization of myelodysplastic syndromes with erythroid predominance from selected publications as compared to our study.

    Table 1.
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Vol 104 Issue 12

Haematologica: 104 (12)
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The non-erythroid myeloblast count rule in myelodysplastic syndromes: fruitful or futile?
Margot F. van Spronsen, Theresia M. Westers, Birgit I. Lissenberg-Witte, Mariëlle Wondergem, Gert J. Ossenkoppele, Arjan A. van de Loosdrecht
Haematologica Dec 2019, 104 (12) e547-e550; DOI: 10.3324/haematol.2018.212563

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Margot F. van Spronsen, Theresia M. Westers, Birgit I. Lissenberg-Witte, Mariëlle Wondergem, Gert J. Ossenkoppele, Arjan A. van de Loosdrecht
Haematologica Dec 2019, 104 (12) e547-e550; DOI: 10.3324/haematol.2018.212563
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