The WHO diagnostic criteria for polycythemia vera-role of red cell mass versus hemoglobin/hematocrit level and morphology - PubMed (original) (raw)

. 2018 Sep;97(9):1581-1590.

doi: 10.1007/s00277-018-3344-3. Epub 2018 May 1.

Mirjana Gotic 2 3, Heinz Gisslinger 4, Ivan Soldatovic 5, Dijana Sefer 1, Mila Tirnanic 6 7, Danijela Lekovic 1, Maja Perunicic Jovanovic 8, Martin Schalling 4, Bettina Gisslinger 4, Christine Beham-Schmid 9, Ingrid Simonitsch-Klupp 10, Jürgen Thiele 11

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The WHO diagnostic criteria for polycythemia vera-role of red cell mass versus hemoglobin/hematocrit level and morphology

Ljubomir Jakovic et al. Ann Hematol. 2018 Sep.

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.

Keywords: Bone marrow morphology; Clinical criteria; Masked polycythemia vera; Polycythemia vera; Red cell mass; WHO classification 2016.

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