Castleman's Disease: Systematic Analysis of 416 Patients from the Literature

Abstract

Background. Castleman’s disease is a rare primary dis- ease of the lymph nodes with limited available clinical information. Methods.Asystematic 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 out- come in univariate analyses. The 3-year disease-free sur- vival (DFS) rate for patients with unicentric hyaline vascular disease (49.5% of cases, class I) was 92.5%, ver- sus 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 multi- centric plasma cell disease and had a 3-year DFS rate of only 27.8%. Kaposi’s sarcoma and lymphoma were ob- served 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, organo- megaly, 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.