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Review
. 2011;16(9):1316-24.
doi: 10.1634/theoncologist.2011-0075. Epub 2011 Jul 17.

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

Nadia Talat  1 , Klaus-Martin Schulte
Affiliations
Review

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

Nadia Talat et al. Oncologist. 2011.

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.

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Conflict of interest statement

Disclosures

Nadia Talat: None; Klaus-Martin Schulte: None.

Section Editor George P. Canellos discloses a consulting relationship with the Celgene Business Advisory Board.

Reviewer “A” discloses a consulting relationship with and research funding received from Merck and Centocor/Ortho Biotech.

The content of this article has been reviewed by independent peer reviewers to ensure that it is balanced, objective, and free from commercial bias. On the basis of disclosed information, all conflicts of interest have been resolved.

Figures

Figure 1.
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.
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.
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.
See this image and copyright information in PMC

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