Castleman's Disease Outcomes by Age
Castleman's disease demonstrates significantly worse outcomes in older patients, with multicentric disease (MCD) occurring more frequently in older individuals and carrying a substantially poorer prognosis than unicentric disease (UCD), which predominantly affects younger adults.
Age-Related Disease Distribution
Unicentric Castleman's disease primarily affects younger patients, while multicentric disease occurs in older populations. 1 The median age at diagnosis across all Castleman's disease types is approximately 37 years, but this masks important age-related differences between disease subtypes. 2
- Unicentric disease typically presents in younger adults (mean age in the 20s-30s), often as an incidental finding in asymptomatic individuals 1
- Multicentric disease occurs in older patients, with a distinct age distribution compared to UCD 1
Survival Outcomes by Age and Disease Type
The 5-year overall survival differs dramatically between disease types, with age being a critical prognostic factor in multicentric disease. 2
Overall Survival Rates
- 5-year overall survival for all Castleman's disease: 80.3% 2
- 5-year overall survival for UCD: 93.6% 2
- 5-year overall survival for MCD: 51.2% 2
Age as an Independent Prognostic Factor
In multicentric Castleman's disease, age ≥40 years is an independent predictor of worse overall survival. 2 Multivariate Cox regression analysis identified older age as one of three independent prognostic factors for OS in MCD patients, along with splenomegaly and hypoalbuminemia. 2
Age-Specific Treatment Considerations
Pediatric patients with Castleman's disease require specialized treatment approaches. 3 Interferon-based therapies and anakinra have shown success in pediatric cases, with corticosteroid monotherapy also reported as effective in isolated pediatric cases. 3 Importantly, direct implementation of Langerhans cell histiocytosis protocols has been unsuccessful in pediatric Castleman's disease. 3
Modern therapies have improved outcomes, with 68% 5-year survival reported with interferon-based treatment, and treatment should be continued indefinitely if tolerated. 3
Mortality and Long-Term Outcomes
Overall mortality in Castleman's disease is 22% at median follow-up of 29 months, with 42% of patients failing first-line therapy. 4 The 2-year survival rate is 88% (95% CI 81-95%) across all idiopathic MCD patients. 4
Older patients face additional risks beyond the primary disease. 2 The association between Castleman's disease and secondary malignancies is significant, with 19% of patients developing a separate malignant disease—substantially higher than age-matched controls (expected 6%). 4
Rare Presentations in Elderly Patients
Castleman's disease in elderly patients (>90 years) is exceptionally rare and may present atypically. 5 A case report of a 91-year-old patient presenting with pleural effusion highlights that CD can occur in very advanced age with non-traditional presentations, requiring high clinical suspicion for diagnosis. 5
Clinical Implications
Age should be factored into treatment intensity and prognostic counseling. Younger patients with UCD have excellent outcomes with surgical excision alone, while older patients with MCD require aggressive systemic therapy. 2 The threshold of age 40 years serves as a critical prognostic cutpoint for MCD patients. 2
Rituximab treatment reduces the incidence of non-Hodgkin lymphoma in Castleman's disease patients, though the risk remains elevated. 3 This is particularly relevant given the 19% rate of secondary malignancies and the need for long-term surveillance every 3-6 months. 3