Castleman's disease: Systematic analysis of 416 patients from the literature

Background. Castleman's disease is a rare primary disease of the lymph nodes with limited available clinical information. Methods. A systematic literature search identified 416 cases amenable to detailed analysis. Results. In HIV ^-patients, centricity, pathology type, the presence of symptoms, gender, and age all predict outcome in univariate analyses. The 3-year disease-free survival (DFS) rate for patients with unicentric hyaline vascular disease (49.5% of cases, class I) was 92.5%, versus 45.7% for those with multicentric plasma cell disease (20.2% of cases, class III) and 78.0% for those with any other combination (22.6% of cases, class II) (p <.0001). HIV ⁺ patients (class IV) exclusively presented with multicentric plasma cell disease and had a 3-year DFS rate of only 27.8%. Kaposi's sarcoma and lymphoma were observed in 59.3% and 9.4% of HIV ⁺ patients and in 2.6% and 3.6% of HIV ^-patients (p <.0001). Paraneoplastic pemphigus and the syndrome of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes were observed exclusively in HIV ^-patients at a rate of 1.3% and 1.8%, respectively. Conclusion. Clinical, pathological, and viral markers allow for the classification of Castleman's disease into groups with markedly different outcomes and disease associations.