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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
Author(s) -
Jeryczynski Georg,
Thiele Jürgen,
Gisslinger Bettina,
Wölfler Albert,
Schalling Martin,
Gleiß Andreas,
Burgstaller Sonja,
BuxhoferAusch Veronika,
Sliwa Thamer,
Schlögl Ernst,
Geissler Klaus,
Krauth MariaTheresa,
Nader Alexander,
Vesely Michael,
SimonitschKlupp Ingrid,
Müllauer Leonhard,
BehamSchmid Christine,
Gisslinger Heinz
Publication year - 2017
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.24788
Subject(s) - leukocytosis , myelofibrosis , medicine , essential thrombocythemia , proportional hazards model , gastroenterology , disease , polycythemia vera , bone marrow
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.