A guideline for the diagnosis and management of

polycythaemia vera
2016 WHO Diagnostic Criteria for PV
• PV diagnosis requires meeting either all 3 major criteria
or the first 2 major criteria and 1 minor criterion
Major criteria 1 Hemoglobin Hematocrit Increased red
>16.5 g/dL (men) >49% (men) cell mass
OR OR
>16 g/dL (women) >48% (women)

2 Bone marrow findings of panmyelosis, hypercellularity, pleomorphic

megakaryocytesa
3 Presence of JAK2 mutation or JAK2 exon 12 mutation
Minor criterion 1 Subnormal serum erythropoietin level
Criterion number 2 (BM biopsy) may not be required in cases where other two major criteria and minor criterion are met.
However, initial myelofibrosis (present in up to 20% of patients) can only be detected by performing a BM biopsy; this finding may
predict a more rapid progression to overt myelofibrosis (post-PV MF).

BM, bone marrow; MF, myelofibrosis; PV, polycythemia vera; WHO, World Health Organization.
Arber DA et al. Blood. 2016:127;2391-405.

www.mpnesimple.ca Effective date: 9/1/2018

Revised WHO Criteria:
Why Lower the Hemoglobin Threshold for PV
• New Hb threshold: 16 g/dL (female); 16.5 g/dL (male)
• Cases with red cell mass >25% of predicted value and
hemoglobin values below the WHO 2008 requirement but
with BM features consistent with PV.
• The identification of 35% of patients with PV-consistent BM
features and lower hemoglobin values (similar thrombosis
rate of PV, but higher risk of transformation to myelofibrosis
and blast phase): masked PV.
• Hct has been consistently shown to perform better in
identifying patients with a raised red cell mass (RCM) than
haemoglobin concentration

Alvarez-Larrán A et al. Haematologica. 2012;97:1704-7; Barbui T et al. Am J Hematol. 2014;89:588-90; Cassinat B et al. Leukemia.
2008;22:452-53; Gianelli U et al. Am J Clin Pathol. 2008;130:336-42; Johansson PL et al. Br J Haematol. 2005;129:701-5; Silver RT et
al. Blood. 2013;122:1881-86.
Investigations: Initial assessment
• clinical history:
1. Drug history (prescribed and recreational).
2. Smoking.
3. Alcohol consumption.
4. Body habitus.
5. Systematic questioning should elicit symptoms related to
other potential secondary causes of erythrocytosis.
6. proportion of patients, who have a clear secondary cause
for their erythrocytosis, may not need any further
investigations.
Investigations
BLOOD:
• FCB:
1. Erythrocytosis.
2. Neutrophilia: Higher in smoker > nonsmoker
3. Thrombocytosis, which are common in JAK2 V617F-positive PV and
part of the criteria for JAK2-negative PV.
• Neutrophilia is defined as >125X109/l in this patient group.
• PBF:
1. Circulating blasts.
2. Leucoerythroblastic features.
3. Monocytosis.
• The above mentioned are indications for B.M assessment.
RENAL & LIVER FUNCTIONS:

1. A number of renal and hepatic diseases can cause erythrocytosis.

2. S.Ca levels should also be determined to exclude a parathyroid
adenoma/carcinoma, which rarely causes secondary erythrocytosis.
 Arterial oxygen saturation (SaO2)/carboxyhaemoglobin
• Tissue hypoxia, 2ry erythrocytosis, identified by using pulse
oximetry in the clinic.
• An SaO2 of <92% has been shown to be associated with an absolute
erythrocytosis.
• 3 situations of hypoxic erythrocytosis: These are will give a normal result
1. CO poisoning.
2. high oxygen affinity haemoglobins.
3. sleep apnoea syndrome.(SAS).
• In those with suspected sleep apnoea (heavy snoring with daytime
somnolence or increased BMI>30 kg/m2), referral should be made to a
respiratory or sleep physician.
Serum ferritin
• Low serum ferritin levels are common in PV patients and iron
deficiency can mask the presentation of PV, giving a misleadingly
low Hct because iron deficiency limits erythropoiesis and
hypochromic microcytosis develops.