It is now nearly two years since the publication of the 2016 revision to the World
Health Organization (WHO) classification of myeloid neoplasms and acute leukemia [
[1]
] with one of the most controversial updates being the revised criteria for the myeloproliferative
neoplasm (MPN) of polycythaemia vera (PV). The new major criteria for PV included
a lowering of the diagnostic haemoglobin level (Hb; >16.5 g/dL in men and > 16.0 g/dL
in women) and haematocrit (HCT; >49% in men and >48% in women) and the inclusion of
bone marrow morphological features, in addition to the presence of a JAK2 V617F or exon 12 mutation. The rationale behind these changes was to capture those
patients with masked PV (mPV) in whom the myeloproliferation is hidden due an iron
deficiency that results in a normal or near normal Hb level or HCT [
[2]
]. Reproducibility of these criteria has been subsequently validated [
[3]
,
[4]
]. One concern with reducing the threshold for Hb and HCT as compared to previous
guideline levels was that these new criteria may lead to unnecessary and costly molecular
investigations in a significant proportion of the healthy population [
- Misawa K.
- Yasuda H.
- Araki M.
- Ochiai T.
- Morishita S.
- Nudejima M.
- et al.
The 2016 WHO diagnostic criteria for polycythemia vera renders an accurate diagnosis
to a broader range of patients including masked polycythemia vera: comparison with
the 2008 WHO diagnostic criteria.
Am J Hematol. 2017; 92: E128-E130
[5]
,
[6]
].Keywords
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References
- The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia.Blood. 2016; 127: 2391-2405
- Masked polycythemia vera (mPV): results of an international study.Am J Hematol. 2014; 89: 52-54
- European LeukemiaNet study on the reproducibility of bone marrow features in masked polycythemia vera and differentiation from essential thrombocythemia.Am J Hematol. 2017; 92: 1062-1067
- The 2016 WHO diagnostic criteria for polycythemia vera renders an accurate diagnosis to a broader range of patients including masked polycythemia vera: comparison with the 2008 WHO diagnostic criteria.Am J Hematol. 2017; 92: E128-E130
- Laboratory investigation of myeloproliferative neoplasms (MPNs): recommendations of the Canadian MPN group.Am J Clin Pathol. 2016; 146: 408-422
- Frequency of polycythemia vera in individuals with normal complete blood cell counts according to the new 2016 WHO classification of myeloid neoplasms.Int J Lab Hematol. 2017; 39: 528-531
- Molecular diagnosis of the myeloproliferative neoplasms: UK guidelines for the detection of JAK2 V617F and other relevant mutations.Br J Haematol. 2013; 160: 25-34
- Referral centre variation in requesting JAK2 V617F mutation analysis for the investigation of a myeloproliferative neoplasm.J Clin Pathol. 2012; 65: 1149-1150
- The 2016 WHO classification and diagnostic criteria for myeloproliferative neoplasms: document summary and in-depth discussion.Blood Cancer J. 2018; 8: 15
Article info
Publication history
Published online: April 02, 2018
Accepted:
March 28,
2018
Received:
March 26,
2018
Identification
Copyright
© 2018 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
