Nadia Talat1, Klaus-Martin Schulte. 1. F.R.C.S., Department of Endocrine Surgery, King's College Hospital, King's Health Partners, Denmark Hill, London, SE5 9RS, UK.
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.
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.
Authors: V Ascoli; M C Sirianni; I Mezzaroma; C M Mastroianni; V Vullo; M Andreoni; P Narciso; C C Scalzo; F Nardi; A Pistilli; F Lo Coco Journal: Ann Diagn Pathol Date: 1999-12 Impact factor: 2.090
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