Management of Elevated Hemoglobin and Hematocrit in an Elderly Male
This elderly male requires immediate diagnostic workup to determine the cause of his erythrocytosis (hemoglobin 18.2 g/dL, hematocrit 56.5%), with therapeutic phlebotomy indicated only if hematocrit exceeds 65% with hyperviscosity symptoms, while the primary focus should be identifying and treating the underlying etiology. 1, 2
Immediate Diagnostic Workup Required
The first priority is confirming true erythrocytosis and identifying its cause through systematic laboratory evaluation:
- Repeat hemoglobin and hematocrit measurements to confirm the elevation, as single measurements are unreliable for establishing diagnosis 2
- Order complete blood count with red cell indices, reticulocyte count, peripheral blood smear, serum ferritin, transferrin saturation, and C-reactive protein as the minimum initial workup 2
- Test for JAK2 mutations (both exon 14 and exon 12) to evaluate for polycythemia vera, which is present in up to 97% of cases 2
- Measure serum erythropoietin levels to differentiate between primary (low EPO) and secondary (high EPO) causes 2, 3
Critical Threshold Assessment
The patient's current hematocrit of 56.5% exceeds the intervention threshold of 54% but does not yet meet criteria for immediate phlebotomy:
- Hematocrit >54% warrants intervention to reduce cardiovascular and thromboembolic risk 1
- Therapeutic phlebotomy is indicated only when hematocrit exceeds 65% with symptoms of hyperviscosity (headache, dizziness, visual disturbances, chest pain) after excluding dehydration 1, 2
- Elevated hematocrit increases blood viscosity, which aggravates vascular disease in coronary, cerebrovascular, and peripheral circulation—particularly dangerous in elderly patients 1
Systematic Evaluation for Secondary Causes
Given the patient is a non-smoker without COPD or sleep apnea, evaluate these specific etiologies:
Hypoxia-Independent Causes to Exclude:
- Malignancies producing erythropoietin: renal cell carcinoma, hepatocellular carcinoma, pheochromocytoma 2
- Testosterone use (prescribed or unprescribed), which causes erythrocytosis in 43.8% of injectable formulations and 15.4% of transdermal preparations 1, 2
- Medications: erythropoietin therapy or other drugs stimulating erythropoiesis 2
Hypoxia-Driven Causes (despite negative history):
- Consider sleep study if nocturnal hypoxemia is suspected, as obstructive sleep apnea produces nocturnal hypoxemia driving erythropoietin production 2
- Evaluate for chronic lung disease not yet diagnosed, as COPD can cause secondary erythrocytosis 2
- Assess altitude exposure, as hemoglobin increases by 0.9 g/dL per 1,000 meters above sea level in men 4
Relative Polycythemia:
- Evaluate hydration status and plasma volume, as dehydration, diuretic use, or stress polycythemia (Gaisböck syndrome) can cause relative erythrocytosis 2
Polycythemia Vera Diagnostic Criteria
If JAK2 mutation is positive, apply WHO diagnostic criteria:
- Major criteria: (1) Elevated hemoglobin (>18.5 g/dL in men) or hematocrit (>55% in men), AND (2) Presence of JAK2 mutation 2
- Minor criteria: Bone marrow biopsy showing hypercellularity, subnormal serum erythropoietin level, or endogenous erythroid colony formation 2
- Diagnosis requires: Both major criteria plus one minor criterion, OR first major criterion plus two minor criteria 2
Management Algorithm Based on Findings
If JAK2 Positive (Polycythemia Vera):
- Refer immediately to hematology for management 2
- Maintain hematocrit strictly below 45% through phlebotomy to reduce thrombotic risk 2
- Initiate low-dose aspirin as the second cornerstone of therapy 2
If JAK2 Negative (Secondary Erythrocytosis):
- Treat the underlying condition: manage newly diagnosed sleep apnea, discontinue causative medications (especially testosterone), treat malignancies producing erythropoietin 2
- For smoker's polycythemia (though patient is non-smoker): resolves with smoking cessation 2
- Monitor closely with serial hemoglobin/hematocrit measurements 2
If No Cause Identified:
- Continue systematic evaluation for occult causes 2
- Consider referral to hematology if hemoglobin >20 g/dL, unexplained splenomegaly, or progressive elevation 2
Specific Management Considerations for Elderly Patients
Age-related factors require special attention:
- Do not adjust hemoglobin thresholds downward for age, as anemia reflects poor health and increased vulnerability to adverse outcomes in older persons, not normal aging 4
- Elderly patients with vascular disease are particularly vulnerable to increased blood viscosity from elevated hematocrit 1
- Patients with cardiovascular risk factors (diabetes, hypertension) require more aggressive monitoring and earlier intervention 1
Critical Pitfalls to Avoid
- Do not perform therapeutic phlebotomy without excluding dehydration first, as this can worsen hemoconcentration 2
- Do not perform repeated routine phlebotomies due to risk of iron depletion, decreased oxygen-carrying capacity, and stroke 2
- If phlebotomy is necessary, replace with equal volume of dextrose or saline to prevent further hemoconcentration 2
- Do not overlook coexisting iron deficiency, which can occur with erythrocytosis and requires cautious supplementation with close monitoring 2
- Do not assume this is "normal aging"—the hemoglobin of 18.2 g/dL significantly exceeds the threshold for anemia diagnosis (<13.5 g/dL in males) and requires investigation 4
Monitoring Strategy Going Forward
- Measure hemoglobin/hematocrit every 1-2 months initially until stable and etiology determined 1
- Use hemoglobin rather than hematocrit for monitoring, as hemoglobin is more accurate and remains stable with sample storage, while hematocrit can falsely increase by 2-4% 2
- Monitor for symptoms of hyperviscosity: headache, dizziness, visual disturbances, chest pain, which would prompt more aggressive intervention 1, 2