1. The WHO diagnostic criteria for polycythemia vera—role of red cell mass versus hemoglobin/hematocrit level and morphology
- Author
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Dijana Sefer, Maja Perunicic Jovanovic, Bettina Gisslinger, Martin Schalling, Jürgen Thiele, Mila Tirnanic, Christine Beham-Schmid, Ljubomir Jakovic, Mirjana Gotic, Heinz Gisslinger, Danijela Lekovic, Ingrid Simonitsch-Klupp, and Ivan Soldatovic
- Subjects
Male ,medicine.medical_specialty ,Erythrocytes ,Hematocrit ,Medical Oncology ,World Health Organization ,Gastroenterology ,Hemoglobins ,03 medical and health sciences ,0302 clinical medicine ,Polycythemia vera ,hemic and lymphatic diseases ,Internal medicine ,medicine ,Humans ,Cell Shape ,Polycythemia Vera ,Aged ,Erythrocyte Volume ,Retrospective Studies ,Hematologic Tests ,Hematology ,medicine.diagnostic_test ,Red Cell ,business.industry ,Retrospective cohort study ,General Medicine ,Middle Aged ,Phlebotomy ,medicine.disease ,030220 oncology & carcinogenesis ,Cohort ,Female ,Hemoglobin ,business ,Biomarkers ,030215 immunology - 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.
- Published
- 2018
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