Beyond Hemoglobin: When and How to Work Up Possible Polycythemia Vera

  • October 2019,
  • cmr.2019.1483;
  • DOI: https://doi.org/10.3121/cmr.2019.1483

Abstract

Background WHO 2017 diagnostic criteria for hemoglobin levels in polycythemia vera (PV) were lowered from 185 g/L to 165 g/L for men and from 165 g/L to 160 g/L for women, but these cutoffs were not designed for screening.

Objectives To assess the value of laboratory and clinical parameters in deciding whether to further pursue a diagnosis of PV. A secondary aim was to explore the diagnostic utility of bone marrow morphology.

Methods We evaluated clinical and laboratory parameters that may be useful when considering further diagnostic work-up, emphasizing PV vs. secondary erythrocytosis (SE). 200 patients with JAK2 V617F testing were classified using WHO criteria.

Results Patients with myeloproliferative neoplasms (MPN) were rarely under age 40 and uncommonly obese (BMI ≥ 30 kg/m2). Current smoking history favored SE. SE patients rarely had a platelet count ≥ 450 × 103/uL. Laboratory parameters suggesting greater PV likelihood were: RBC > 6.8 × 106 for men or > 5.9 × 106 for women; low erythropoietin; and low MCV or low ferritin. Bone marrow morphology (available in 111 cases) was generally more cellular in PV vs. SE and assessed disease progression.

Conclusions Readily accessible clinical and laboratory data can assist in considering a PV workup, and a possible diagnostic algorithm is presented. These preliminary findings warrant larger studies to develop a more formal PV-risk scoring system with optimal cutoffs and weighting.

  • Received January 17, 2019.
  • Revision received June 13, 2019.
  • Revision received August 1, 2019.
  • Accepted August 20, 2019.
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