Multicentric Castleman's disease with abundant IgG4-positive cells: a clinical and pathological analysis of six cases

Autor: Yasuharu Sato, Katsuyoshi Takata, Haruka Iwao, Masaru Kojima, Hideyuki Shiomi, Takehiro Tanaka, Tadashi Yoshino, Dai Inoue, Toshiaki Morito, Kohichi Mizobuchi
Rok vydání: 2010
Předmět:
Zdroj: Journal of Clinical Pathology. 63:1084-1089
ISSN: 0021-9746
DOI: 10.1136/jcp.2010.082958
Popis: Background Differentiation between multicentric Castleman9s disease and systemic immunoglobulin (Ig) G4-related lymphadenopathy is sometimes difficult. It has been suggested that measurement of the IgG4-/IgG-positive cell ratio is useful for the differential diagnosis of the two diseases. However, the authors present a detailed report of six patients with multicentric Castleman9s disease with abundant IgG4-positive cells (IgG4-/IgG-positive cell ratio, >40%). Results In the present series, the patients showed systemic lymphadenopathy, polyclonal hypergammaglobulinaemia and elevated serum interleukin-6 (IL-6) and C-reactive protein levels. Further, anaemia, hypoalbuminaemia, hypocholesterolaemia and thrombocytosis were observed. These findings were consistent with those of multicentric Castleman9s disease. Although five patients showed elevated serum IgG4 levels, only two patients showed an increased serum IgG4/IgG ratio. However, the two patients showed highly elevated serum IgG4 levels, but the serum IgG4/IgG ratios were, although increased, not very high. Also, a patient with increased serum IgG4/IgG ratio showed a good response to antihuman IL-6 receptor monoclonal antibody (tocilizumab). Histologically, the germinal centres were mostly small and regressive, and frequently penetrated by hyalinised blood vessels, and there was no eosinophil infiltration. These findings were different from those of IgG4-related lymphadenopathy. Conclusions The authors conclude that multicentric Castleman9s disease sometimes occurs with abundant IgG4-positive cells and elevated serum IgG4 levels. Therefore, the two diseases cannot be differentially diagnosed by immunohistochemical staining alone. Laboratory findings, especially IL-6 level, C-reactive protein level and platelet count, are important for the differential diagnosis of the two diseases.
Databáze: OpenAIRE