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

Abstract

Abstract Learning Objectives After completing this course, the reader will be able to: Describe the centricity and histopathology profiles of each of the three classes of HIV-negative patients identified in this analysis.Correlate nosological classification and outcomes in patients with Castleman's disease. CME This article is available for continuing medical education credit at CME.TheOncologist.com 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.