Jakovic, Ljubomir ORCID: 0000-0003-2400-0112, Gotic, Mirjana ORCID: 0000-0002-8590-0328, Gisslinger, Heinz, Soldatovic, Ivan, Sefer, Dijana, Tirnanic, Mila, Lekovic, Danijela ORCID: 0000-0002-6194-8298, Jovanovic, Maja Perunicic, Schalling, Martin, Gisslinger, Bettina, Beham-Schmid, Christine, Simonitsch-Klupp, Ingrid and Thiele, Juergen (2018). The WHO diagnostic criteria for polycythemia vera-role of red cell mass versus hemoglobin/hematocrit level and morphology. Ann. Hematol., 97 (9). S. 1581 - 1591. NEW YORK: SPRINGER. ISSN 1432-0584

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Abstract

Regarding diagnosis of polycythemia vera (PV), discussion persists about hemoglobin (Hb) and/or hematocrit (Hct) threshold values as surrogate markers for red cell mass (RCM) and the diagnostic impact of bone marrow (BM) morphology. We performed a retrospective study on 290 patients with PV (151 males, 139 females; median age 65 years) presenting with characteristic BM features (initial biopsies, centralized evaluation) and endogenous erythroid colony (EEC) formations. This cohort included (1) a group of 229 patients when following the 2008 versus 256 patients diagnosed according to the 2016 World Health Organization (WHO) guidelines, all presented with increased RCM; (2) masked PV patients with low Hb (n = 143)/Hct (n = 45) recruited from the 2008 WHO cohort; (3) a cohort of 17 PV patients with elevated diagnostic Hb/Hct levels but low RCM; and (4) nine PV patients with increased RCM, opposing low Hb/Hct values. All patients were treated according to current PV guidelines (phlebotomies 87%, hydroxyurea 79%, and acetylsalicylic acid 87%). Applying the 2016 WHO criteria significantly increased concordance between RCM and Hb values compared with the 2008 WHO criteria (90 vs. 43% in males and 83 vs. 64% in females). Further analysis of the WHO 2016 PV cohort revealed that increased RCM is associated with increased Hb/Hct (93.8/94.6%). Our study supports and extends the diagnostic impact of the 2016 revised WHO classification for PV by highlighting the importance of characteristic BM findings and implies that Hb/Hct threshold values may be used as surrogate markers for RCM measurements.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Jakovic, LjubomirUNSPECIFIEDorcid.org/0000-0003-2400-0112UNSPECIFIED
Gotic, MirjanaUNSPECIFIEDorcid.org/0000-0002-8590-0328UNSPECIFIED
Gisslinger, HeinzUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Soldatovic, IvanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sefer, DijanaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Tirnanic, MilaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lekovic, DanijelaUNSPECIFIEDorcid.org/0000-0002-6194-8298UNSPECIFIED
Jovanovic, Maja PerunicicUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schalling, MartinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gisslinger, BettinaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Beham-Schmid, ChristineUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Simonitsch-Klupp, IngridUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Thiele, JuergenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-175799
DOI: 10.1007/s00277-018-3344-3
Journal or Publication Title: Ann. Hematol.
Volume: 97
Number: 9
Page Range: S. 1581 - 1591
Date: 2018
Publisher: SPRINGER
Place of Publication: NEW YORK
ISSN: 1432-0584
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
WORLD-HEALTH-ORGANIZATION; ESSENTIAL THROMBOCYTHEMIA; MYELOPROLIFERATIVE NEOPLASMS; MYELOID NEOPLASMS; ACUTE-LEUKEMIA; CLASSIFICATION; REVISION; REPRODUCIBILITY; MANAGEMENT; PROPOSALMultiple languages
HematologyMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/17579

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