Jeryczynski, Georg, Thiele, Juergen, Gisslinger, Bettina, Woelfler, Albert, Schalling, Martin, Gleiss, Andreas, Burgstaller, Sonja, Buxhofer-Ausch, Veronika, Sliwa, Thamer, Schloegl, Ernst, Geissler, Klaus, Krauth, Maria-Theresa, Nader, Alexander ORCID: 0000-0001-5987-2397, Vesely, Michael, Simonitsch-Klupp, Ingrid, Muellauer, Leonhard, Beham-Schmid, Christine and Gisslinger, Heinz (2017). 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., 92 (9). S. 885 - 892. HOBOKEN: WILEY. ISSN 1096-8652

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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.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Jeryczynski, GeorgUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Thiele, JuergenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gisslinger, BettinaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Woelfler, AlbertUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schalling, MartinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gleiss, AndreasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Burgstaller, SonjaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Buxhofer-Ausch, VeronikaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sliwa, ThamerUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schloegl, ErnstUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Geissler, KlausUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Krauth, Maria-TheresaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Nader, AlexanderUNSPECIFIEDorcid.org/0000-0001-5987-2397UNSPECIFIED
Vesely, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Simonitsch-Klupp, IngridUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Muellauer, LeonhardUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Beham-Schmid, ChristineUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gisslinger, HeinzUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-221187
DOI: 10.1002/ajh.24788
Journal or Publication Title: Am. J. Hematol.
Volume: 92
Number: 9
Page Range: S. 885 - 892
Date: 2017
Publisher: WILEY
Place of Publication: HOBOKEN
ISSN: 1096-8652
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
CHRONIC IDIOPATHIC MYELOFIBROSIS; BONE-MARROW BIOPSY; MYELOPROLIFERATIVE NEOPLASMS; HISTOLOGICAL CRITERIA; POLYCYTHEMIA-VERA; WORKING GROUP; REPRODUCIBILITY; CLASSIFICATION; FIBROSIS; REVISIONMultiple languages
HematologyMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/22118

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