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

Abstract

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.

Figure 1.

Age distribution in 384 HIV⁻ patients. (A): Grouped by decade: overall median age, 37 years. (B): Grouped by centricity: UCD median age, 30 years; MCD median age, 52 years. (C): Grouped by gender: female median age, 33 years; male median age, 38 years. (D): Grouped by histopathology type: HV median age, 30 years; PC median age, 41 years. Abbreviations: HV, hyaline vascular; MCD, multicentric disease; PC, plasma cell; UCD, unicentric disease.

Figure 2.

Centricity, histopathology type, gender, and age influence the 3-year disease-free survival rate in a Kaplan–Meier analysis of 148 HIV⁻ patients. Abbreviations: HV, hyaline vascular; MCD, multicentric disease; PC, plasma cell; UCD, unicentric disease.

Figure 3.

Variation in the 3-year disease-free survival rate in a Kaplan–Meier analysis of 166 patients depending on nosological classification. (A): Only HIV⁻ patients were included (n = 148). Class I, HV UCD; class II, PC UCD or mixed pathology UCD or HV MCD; class III, PC MCD. (B): Additionally includes HIV⁺ patients (n = 166). Abbreviations: HV, hyaline vascular; MCD, multicentric disease; PC, plasma cell; UCD, unicentric disease.