Medizinische Universität Graz - Research portal
Selected Publication:
SHR Neuro Cancer Cardio Lipid Metab Microb
Jeryczynski, G; Thiele, J; Gisslinger, B; Wölfler, A; Schalling, M; Gleiß, A; Burgstaller, S; Buxhofer-Ausch, V; Sliwa, T; Schlögl, E; Geissler, K; Krauth, MT; Nader, A; Vesely, M; Simonitsch-Klupp, I; Müllauer, L; Beham-Schmid, C; Gisslinger, H.
Pre-fibrotic/early primary myelofibrosis vs. WHO-defined essential thrombocythemia: The impact of minor clinical diagnostic criteria on the outcome of the disease.
Am J Hematol. 2017; 92(9):885-891
Doi: 10.1002/ajh.24788
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- Co-authors Med Uni Graz
- Beham-Schmid Christine
- Wölfler Albert
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- Abstract:
- The 2016 revised WHO criteria for the diagnosis of pre-fibrotic/early primary myelofibrosis (pre-PMF) require at least one of the following four borderline expressed minor clinical criteria: anemia, leukocytosis, elevated lactate dehydrogenase and splenomegaly. In this study, we evaluated the relative frequency of these four criteria in a group of 170 pre-PMF patients and compared them to 225 ET cases. More than 91% of pre-PMF cases showed one or more of these features required for diagnosis, by contrast with only 48% of ET patients. According to clinical data the cumulative risk of progression to advanced/overt PMF in pre-PMF was 36.9% after 15 years. After fitting cox regression models to analyze the impact of the minor criteria on overall survival, only leukocytosis remained as a significant predictor of survival in both pre-PMF and ET. Molecular characterization showed differences in survival in pre-PMF but not ET, with CALR being a more favorable mutation than JAK2. The different outcome of pre-PMF versus ET and associated molecular genetic data supports the concept of two different entities, rather than a continuum of the same disease. Although slightly less than 50% of ET patients also show one or more minor clinical criteria, accurate distinction between ET and pre-PMF is possible by following an integrated approach including histomorphological diagnosis and presence of minor clinical criteria. © 2017 Wiley Periodicals, Inc.
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