with great interest we have read the recent article by Ernest et al. [
[1]
] reporting distinctive clinical and laboratory features of patients with Janus Kinase
2 (JAK2)-unmutated erythrocytosis. Considering that discriminating polycythemia vera
(PV) patients from JAK2-unmutated patients is of major importance in everyday clinical
practice, especially due to different treatment approaches, the study by Ernest et al.
[
[1]
] provides valuable information for the practicing clinicians. It should be emphasized
again that laboratory thresholds required for investigation of PV, a clonal hematopoietic
stem cell disorder characterized by an increased thrombotic risk, have been significantly
lowered in 2016 by the World Health Organization (WHO) [
[2]
], causing a substantial rise in PV investigations worlwide. In fact, one Canadian
study reported that approximately 4% of all male subjects would need PV workup based
solely on their hemoglobin and hematocrit values [
[3]
]. In addition, subnormal erythropoietin levels, a minor diagnostic criterion by the WHO, may not be fully reliable, considering that approximately
one-third of patients with PV can have erythropoietin levels within the reference
range, which is common in obese patients, smokers, or in those with chronic obstructive
pulmonary disorder (COPD) [
[4]
]. This, in turn, may cause an increase in bone marrow examinations which are certainly
painful for the patients. In fact, recent reports suggest that 31%–41.5% of JAK2-unmutated
patients undergo (often unnecessary) diagnostic bone marrow biopsies [
[5]
,
[6]
]. Finally, red blood cell mass measurement using the radioactive isotopes is considered
to be the most accurate diagnostic test for PV [
[7]
] but it is expensive, toxic, and unavailable in most countries.To read this article in full you will need to make a payment
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References
- Characteristics of JAK2 unmutated erythrocytosis: distinctive traits between polycythemia vera and non-polycythemia vera patients.Eur J Intern Med. 2022; (S:S0953-6205(22)00319-3)
- The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia.Blood. 2016; 127: 2391-2405
- Laboratory investigation of myeloproliferative neoplasms (MPNs): recommendations of the canadian mpn group.Am J Clin Pathol. 2016; 146: 408-422
- The role of a low erythropoietin level for the polycythemia vera diagnosis.Blood Cells Mol Dis. 2020; 80102355
- Phenotypical differences and thrombosis rates in secondary erythrocytosis versus polycythemia vera.Blood Cancer J. 2021; 11: 75
- Thrombotic risk in secondary polycythemia resembles low-risk polycythemia vera and increases in specific subsets of patients.Thromb Res. 2022; 209: 47-50
- Time for revival of the red blood cell count and red cell mass in the differential diagnosis between essential thrombocythemia and polycythemia vera?.Haematologica. 2019; 104: 2119-2125
- Erythrocytosis in the general population: clinical characteristics and association with clonal hematopoiesis.Blood Adv. 2020; 4 (9): 6353-6363
- High platelet-to-lymphocyte ratio may differentiate polycythemia vera from secondary polycythemia.Wien Klin Wochenschr. 2022; 134 (202): 483-486
- Neutrophil-to-lymphocyte and platelet-to-lymphocyte ratios as prognostic biomarkers in polycythemia vera.Int J Lab Hematol. 2022; 44: e145-e148
Article info
Publication history
Published online: September 29, 2022
Accepted:
September 26,
2022
Received:
September 13,
2022
Identification
Copyright
© 2022 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
