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

Abstract

Background: World Health Organization (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: The primary aim of this study was 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). We classified 200 patients with JAK2 V617F testing using WHO criteria. Results: Patients with myeloproliferative neoplasms (MPN) were rarely under age 40 and uncommonly obese (BMI ≥ 30 kg/m 2). Current smoking history favored SE, and these patients rarely had a platelet count ≥ 450 x 10 3 /uL. Laboratory parameters suggesting greater PV likelihood were: RBC > 6.8 x 10 6 for men or > 5.9 x 10 6 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.

Phenotypical differences and thrombosis rates in secondary erythrocytosis versus polycythemia vera

“…While the classic coupling of JAK2-positive/subnormal serum erythropoietin (Epo) greatly increases the likelihood of PV diagnosis 4,5 , those not strictly fulfilling these criteria represent a heterogeneous population for whom a systematic approach has been difficult to establish 6 . Though efforts have been made to operationally discriminate between the various forms of erythrocytosis, data comparatively assessing SE and PV populations are scarce 7,8 . These support different clinical profiles 7,8 , while reports of outcomes, including thrombosis, have been inconsistent 9,10 .…”

“…Though efforts have been made to operationally discriminate between the various forms of erythrocytosis, data comparatively assessing SE and PV populations are scarce 7,8 . These support different clinical profiles 7,8 , while reports of outcomes, including thrombosis, have been inconsistent 9,10 . Furthermore, little information exists on how these populations are managed in a real world setting, and it may be speculated that SE cohorts are subject either to under or over investigating and treatment.…”

Polycythaemia vera: molecular genetics, diagnostics and therapeutics

“…The characteristic thrombocytosis for juveniles seen in ET presents at the mean, 1·109 × 10 9 l −1 compared to the significantly lower counts seen in juvenile PV in the range 207–394 × 10 9 l −1 [2]. With respect to platelet counts in adults, higher median counts of 811 × 10 9 l −1 are reported in ET compared to 686 × 10 9 l −1 seen in PV [30]. Across the broad spectrum of age for PV patients, these data suggest a general upward trend of the platelet levels between youth through adulthood.…”

The restrictive type and infectious complications might predict nadir hematological values among individuals with anorexia nervosa during the refeeding period: a retrospective study

“…Thrombocytopenia (platelets < 100 ×10 3 /μl) was seen in 16.8 % of patients (17 of 101 admissions). Although the hemoglobin levels at admission in 4 patients were > 16g/dl, their hemoglobin levels during the refeeding period decreased to < 13.2g/dl, suggesting that these patients had elevated hemoglobin levels upon admission, but not polycythemia, for which hemoglobin level for women is > 16g/dl [33]. Red blood cell transfusion was used to treat 12 admissions (11.9%) from 11 individual patients whose average nadir hemoglobin value was 5.1 g/dl (range: 1.7-7.4 g/dl).…”