Multicentric Castleman's disease with abundant IgG4-positive cells: a clinical and pathological analysis of six cases
Multicentric Castleman's disease with abundant IgG4-positive cells: a clinical and pathological analysis of six cases
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DOI：
10.1136/jcp.2010.082958
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发表时间：
2010-12-01
影响因子：
3.4
通讯作者：
Yoshino, Tadashi
中科院分区：
文献类型：
--
作者：
Sato, Yasuharu;Kojima, Masaru;Yoshino, Tadashi
背景：多中心性Castleman病和全身性免疫球蛋白(Ig)G4相关淋巴结病有时很难区分。提示测定IgG4/Ig G阳性细胞比率有助于两种疾病的鉴别诊断。然而，作者详细报道了6例多中心Castleman病患者，其中IgG4阳性细胞丰富(IgG4/Ig G阳性细胞比率，40%)。结果本组患者表现为全身性淋巴病变、多克隆高丙种球蛋白血症和血清白介素6(IL-6)和C反应蛋白水平升高。此外，还观察到贫血、低蛋白血症、低胆固醇血症和血小板增多症。这些发现与多中心Castleman病的结果一致。虽然有5名患者的血清IgG4水平升高，但只有两名患者的血清IgG4/Ig G比值升高。两例患者血清IgG4水平明显升高，但血清IgG4/Ig G比值虽有所升高，但并不是很高。此外，1例血清IgG4/Ig G比值升高的患者对抗人IL-6受体单抗(Tocilizumab)有良好的反应。组织学上，生发中心大多较小且退化，常被透明血管穿透，未见嗜酸性粒细胞浸润。结论多中心性Castleman病的发病常伴有丰富的IgG4阳性细胞和血清IgG4水平升高。因此，仅凭免疫组织化学染色不能鉴别诊断这两种疾病。实验室检查结果，尤其是IL-6水平、C反应蛋白水平和血小板计数，对这两种疾病的鉴别诊断具有重要意义。
Background Differentiation between multicentric Castleman's 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 Castleman's 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 Castleman's 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 Castleman's 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.