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Myelodysplastic syndromes with bone marrow fibrosis: a myelodysplastic disorder with proliferative features

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Summary

We report on 22 patients with myelodysplastic syndrome (MDS), all of whom showed striking marrow fibrosis. Variable blood counts, often with teardrop poikilocytosis and a leukoerythroblastic picture, were present at diagnosis. Visceral enlargement was detected in 17 patients with a distinct splenomegaly in seven cases. All cases demonstrated dysplasia in at least two cell lineages. No specific cytogenetic abnormality seems to characterize this group of patients. Southern blot analysis showed no breakpoint cluster region rearrangement as observed in classical chronic myeloid leukemia. Ferrokinetic studies revealed quantitatively deficient erythropoiesis in all except two cases and an abnormally high fraction of ineffective erythropoiesis in all. Splenic erythropoiesis was present in eight patients. The median survival was 18 months. At the time of this report, 12 patients had died. The causes of death were disease progression (7 patients) and infection (5 patients). One might speculate that the present series of cases represents a transition between MDS and myeloproliferative disease, thereby dysplaying characteristics of both groups of diseases.

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Authors and Affiliations

  1. Department of Hematology, University Hospital Gasthuisberg, Herestraat 49, 3000, Leuven, Belgium

    G. E. G. Verhoef, P. Zacheé & M. A. Boogaerts

  2. Department of Pathology, University Hospital Gasthuisberg, Herestraat 49, 3000, Leuven, Belgium

    C. De Wolf-Peeters & S. Deprez

  3. Center for Human Genetics, University Hospital Gasthuisberg, Herestraat 49, 3000, Leuven, Belgium

    P. Meeus, M. Stul, J. J. Cassiman & H. Van den Berghe

  4. Department of Hematology, Cliniques Universitaires St-Luc, Avenue Hippocrate 10, B-12-Brussels, Belgium

    A. Ferrant

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  1. G. E. G. Verhoef
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  2. C. De Wolf-Peeters
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  5. P. Meeus
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  9. H. Van den Berghe
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Additional information

This work was supported in part by a grantactie levenslijn no. 7004190

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Verhoef, G.E.G., De Wolf-Peeters, C., Ferrant, A. et al. Myelodysplastic syndromes with bone marrow fibrosis: a myelodysplastic disorder with proliferative features. Ann Hematol 63, 235–241 (1991). https://doi.org/10.1007/BF01698371

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  • DOI: https://doi.org/10.1007/BF01698371

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