1. ¹Institut für Pathologie, Medizinische Hochschule Hannover, Hannover, Germany
2. ²Institut für Pathologie, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany
3. ³Klinik für Hämatologie, Hämostaseologie und Onkologie, Medizinische Hochschule Hannover, Hannover, Germany
4. ⁴Institut für Biometrie, Medizinische Hochschule Hannover, Hannover, Germany
5. ⁵Institut für Zell- und Molekularpathologie, Medizinische Hochschule Hannover, Hannover, Germany
6. ⁶Institut für Pathologie, Universität Bern, Bern, Switzerland

Correspondence: Dr med. G Buesche, Institut für Pathologie, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, Hannover D-30625, Germany. E-mail: guntram.buesche@arcor.de

Received 31 July 2007; Revised 9 October 2007; Accepted 10 October 2007; Published online 22 November 2007.

Abstract

Marrow fibrosis (MF) has rarely been studied in myelodysplastic syndromes (MDS). There are no data on occurrence and significance of MF in the context of the World Health Organization (WHO) classification of disease. In total, 349 bone marrow biopsies from 200 patients with primary MDS were examined for MF and its prognostic relevance. MF correlated with multilineage dysplasia, more severe thrombopenia, higher probability of a clonal karyotype abnormality, and higher percentages of blasts in the peripheral blood (P<0.002). Its frequency varied markedly between different MDS types ranging from 0 (RARS) to 16% (RCMD, RAEB, P<0.007). Two patients with MF showed a Janus kinase-2 mutation (V617F). Patients with MF suffered from marrow failure significantly earlier with shortening of the survival time down to 0.5 (RAEB-1/-2), and 1–2 (RCMD, RA) years in median (P<0.00005). The prognostic relevance of MF was independent of the International Prognostic Scoring System and the classification of disease. Conclusion: The risk of MF Differs markedly between various subtypes of MDS. MF indicates an aggressive course with a significantly faster progression to fatal marrow failure and should therefore be considered in diagnosis, prognosis and treatment of disease.