The immunophenotypic patterns of follicle centre and mantle zone in Castleman's disease

Castleman's disease is an uncommon disease and the histopathogenesis is poorly understood. This study aims to investigate their clinicopathological and immunophenotypic profile.

Castleman's disease was reconfirmed in biopsy tissue from 10 patients in a period of 17 years. Immunohistochemical staining was performed on the archival materials, with antibodies to lymphoid antigens and oncogene products, Bcl-6 and Bcl-2. Six reactive hyperplastic lymph nodes and three tonsils were used for comparative study of the phenotypic expression.

There were three cases of plasma-cell and seven of hyaline-vascular variant. The ages of patients ranged from eight to 60 years (median 30 years). The three patients with plasma-cell variant were older, all females. Two of the plasma-cell variant had multicentric lesions associated with systemic disease. All patients with hyaline-vascular variant had localised disease. Atrophic follicle centres were present in all the diagnostic tissue of both subtypes, with loss of Bcl-6 follicular centre B-cells and presence of relatively few CD57 T-cells. These follicle centres stained strongly with anti-CD21. In the mantle zone, CD5 expression was observed in only two cases and Bcl-2 expression similar to reactive follicles was present in six.

Loss of Bcl-6 B-cells in the atrophic follicle centres, characteristic CD21 network patterns, low rate of CD5 and Bcl-2 expression in the mantle-zone lymphocytes are present in both variants of Castleman's disease, differ distinctly from reactive follicles. The phenotypic similarity in these two variants suggests possibility of closely related pathogeneses.