Polycythemia Vera Onset in Adulthood
Yes, polycythemia vera (PV) characteristically presents in adulthood, with a median age at diagnosis of approximately 60 years. 1
Age Distribution at Diagnosis
- The median age at diagnosis is approximately 60 years, establishing PV as predominantly an adult-onset disease 1
- Approximately 7% of patients are diagnosed before age 40 years, indicating that while uncommon, younger adult onset does occur 1
- Children are rarely diagnosed with PV, making pediatric presentation exceptional rather than typical 1
- The disease shows a slight male preponderance with a 1.2:1 ratio 1
Risk Stratification by Age
The Mayo Clinic Proceedings guidelines explicitly incorporate age into risk stratification, underscoring the adult nature of this disease:
- Low-risk disease is defined as age younger than 60 years with no history of thrombosis 1
- High-risk disease includes age 60 years or older OR a positive history of thrombosis, making advanced age itself a major risk factor 1
- Age >60 years is independently associated with worse survival outcomes 2
Clinical Implications of Adult Onset
Treatment strategies differ based on age at presentation:
- Younger patients (<60 years) without thrombosis history may be managed with phlebotomy alone plus low-dose aspirin 1
- In younger high-risk patients, interferon-α is preferred over hydroxyurea or busulfan due to concerns about long-term leukemogenicity, though evidence for this concern remains limited 1
- Women of childbearing age should receive interferon-α rather than hydroxyurea or busulfan due to theoretical teratogenicity risk 1
- Elderly patients (>60 years) with life expectancy <10 years may be candidates for radioactive phosphorus (³²P) if compliance is an issue 1
Survival and Long-Term Outcomes
- Median survival is approximately 15 years overall but exceeds 35 years for patients aged ≤40 years at diagnosis 2, 3
- Twenty-year risk of transformation to myelofibrosis is approximately 16%, and to acute leukemia is 4% 2, 3
- The risk of disease transformation increases progressively beyond the first decade 1
Common Pitfall to Avoid
Do not dismiss the diagnosis of PV in younger adults (age <40 years) - while only 7% of cases present before age 40, these patients have markedly better prognosis with median survival exceeding 35 years, making accurate diagnosis and appropriate long-term management critical 1, 2