Diagnostic Hemoglobin, Hematocrit, and RBC Values for Polycythemia Vera
For polycythemia vera diagnosis, hemoglobin ≥18.5 g/dL in men or ≥16.5 g/dL in women, or hematocrit >52% in men or >48% in women, should trigger diagnostic evaluation including JAK2 mutation testing. 1
Primary Diagnostic Thresholds
Hemoglobin Criteria
- Men: Hemoglobin ≥18.5 g/dL 2, 1
- Women: Hemoglobin ≥16.5 g/dL 2, 1
- Alternative: Hemoglobin >99th percentile of method-specific reference range adjusted for age, sex, and altitude 2
Hematocrit Criteria
- Men: Hematocrit >52% 1, 3
- Women: Hematocrit >48% 3
- When hematocrit ≥60%, absolute erythrocytosis is virtually always present 4
Red Cell Mass
Important Diagnostic Nuances
Lower Threshold Considerations
A sustained increase in hemoglobin of ≥2 g/dL from an individual's baseline (not attributable to iron deficiency correction) may indicate PV even without meeting absolute thresholds. 2, 1 This captures early or "masked" polycythemia vera cases that would otherwise be missed 2.
Masked Polycythemia Vera
- Approximately 35% of PV patients present with hemoglobin below the traditional WHO thresholds (16.0-18.4 g/dL in men, 15.0-16.4 g/dL in women) but still have true disease 3
- These patients more frequently present with thrombocytosis, increased bone marrow reticulin fibrosis, and worse overall survival 3
- When hematocrit is 48-52% in men or 48% in women with JAK2 mutation and other PV features, proceed with full diagnostic workup 3, 5
Critical Diagnostic Algorithm
Step 1: Initial Screening
Suspect PV when:
- Hemoglobin/hematocrit exceeds 95th percentile for sex and race 2
- Documented sustained increase in hemoglobin above individual baseline 2
- Borderline-high hematocrit with PV-related features (thrombocytosis, leukocytosis, microcytosis from iron deficiency, splenomegaly, aquagenic pruritus, unusual thrombosis, erythromelalgia) 2
Step 2: Confirmatory Testing
- JAK2 mutation testing (present in >95% of PV cases) 2
- Serum erythropoietin level (typically low in PV, specificity >90%) 2
- Bone marrow biopsy showing hypercellularity with trilineage growth (panmyelosis) 2, 1
Step 3: Red Cell Mass Measurement
Red cell mass measurement should be performed when: 5
- Hematocrit is 48-52% in men or 48% in women with clinical suspicion 5
- Portal vein thrombosis, isolated leukocytosis, thrombocytosis, or splenomegaly present with borderline hematocrit 6
Red cell mass measurement is NOT needed when: 2
- Hematocrit >60% without obvious hemoconcentration (RCM almost always elevated) 2
- Hemoglobin clearly exceeds diagnostic thresholds 4
Common Pitfalls and Caveats
Iron Deficiency Masking
Iron deficiency can lower hemoglobin/hematocrit and mask true polycythemia. 2, 1 In routine clinical practice, you can make a working diagnosis of PV despite not meeting WHO hemoglobin criteria if iron deficiency is present, but for research purposes, demonstrate criteria fulfillment after iron replacement 2.
Hemoglobin vs. Hematocrit Discordance
- Hemoglobin >18.5 g/dL in men has high specificity (predicts absolute erythrocytosis in only 35% of male PV patients due to low sensitivity) 4
- Hematocrit shows better diagnostic accuracy than hemoglobin 5
- Hematocrit ≥60% always indicates absolute erythrocytosis in both sexes 4
Plasma Volume Expansion
Increased plasma volume (average +36% in masked PV vs. +9.5% in overt PV) can mask elevated red cell mass, keeping hemoglobin and hematocrit in normal range despite true polycythemia 6. This defines "inapparent polycythemia vera" affecting approximately 17% of PV patients 6.