Pre-fibrotic/early primary myelofibrosis vs. WHO-defined essential thrombocythemia: The impact of minor clinical diagnostic criteria on the outcome of the disease
In: American Journal of Hematology, Jg. 92 (2017-06-09), S. 885-891
Online
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Zugriff:
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.
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Pre-fibrotic/early primary myelofibrosis vs. WHO-defined essential thrombocythemia: The impact of minor clinical diagnostic criteria on the outcome of the disease
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Autor/in / Beteiligte Person: | Vesely, Michael ; Burgstaller, Sonja ; Simonitsch-Klupp, Ingrid ; Nader, Alexander ; Müllauer, Leonhard ; Krauth, Maria-Theresa ; Gleiß, Andreas ; Geissler, Klaus ; Schlögl, Ernst ; Schalling, Martin ; Gisslinger, Heinz ; Buxhofer-Ausch, Veronika ; Thiele, Juergen ; Beham-Schmid, Christine ; Wölfler, Albert ; Jeryczynski, Georg ; Sliwa, Thamer ; Gisslinger, Bettina |
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Zeitschrift: | American Journal of Hematology, Jg. 92 (2017-06-09), S. 885-891 |
Veröffentlichung: | Wiley, 2017 |
Medientyp: | unknown |
ISSN: | 0361-8609 (print) |
DOI: | 10.1002/ajh.24788 |
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