Castleman病7例临床病理分析

Castleman's disease:7 cases report and review of literature

目的探讨Castleman病的临床诊断鉴别诊断及病理组织学特征。方法通过组织学、组织化学、免疫组化及基因重排克隆性分析等方法对Castleman病进行研究,并结合文献加以分析。结果7例Castleman病中5例为透明血管型,表现为增生的淋巴结内均匀的分布着大小相近的小淋巴滤泡;滤泡生发中心变小,其内可见泡状核的滤泡树突状细胞,有透明变性的小动脉穿入;外套层明显增厚,小淋巴细胞呈同心圆排列于血管周围,形成特征性的“洋葱”样同心圆结构,类似胸腺小体;滤泡间毛细血管增加,可有玻璃样变和纤维化;淋巴窦大部分消失或全部消失。2例浆细胞型特点是滤泡间大量的成熟浆细胞增生,有淋巴窦消失改变,滤泡内的毛细血管穿入,“洋葱”样改变不明显,滤泡间可见PAS染色阳性的无定型的嗜酸性物质沉积。其中1例浆细胞型部分区域淋巴细胞显著增生,弥漫成片,免疫组化显示κ链限制性表达,基因重排克隆性分析显示IG受体阳性,表明已转化为B细胞淋巴瘤。结论Castleman病是一种特殊类型的淋巴结增生性疾病,可发生于任何年龄,诊断时要与反应性滤泡性增生、淋巴瘤等进行鉴别,并注意是否发生淋巴瘤等恶性转化。

Objective To study the clinical and pathologic features as well as the differential diagnosis Of Castleman disease. Histological appearances and immnunohistochemisty and gene rearrangement analysis were applied to analyze 7 cases of Caatemon disease with review Of literatures, Results 5 in 7 cases of Castleman diseases were hyaline-vascular type or angiOfollicular, which showed small follicles scattered in a mass Of lymphoid tissue. The follicles showed marked vascular proliferation and hyalinization of their abnormal germinal centers. The apoarance corresponded to that of progressively transformed germinal centers. Many Of the large cells with vesicular nuclei presented in the hyaline center were follicular dentritic cells. There was a fight concentric laying Of lymphocytes at the periphery of follicles （corresponding to the mantle zone ）, resulting in an onionskin appearance. The interfollicular stroma was also prominent, with numerous hyperplastic veasles of the postcapiUary venule type and an admixture Of lymphocyte, plasma cells and eosinophls. Sinuses were characteristically absent. The other 2 cases were plasma cell type that characterized by diffuse plasma cell proliferation in the interfollicular tissue. The hyaline-vascular changes in the follicles were inconspicuous or absent; instead, one often encountered in the center Of these follicles a deposition Of an amorphous acidophilic material that probably contained fibrin and immune complexs. One case of Casdeman disease Of plasma cell type showed significant lymphocytic proliferation. Restricted expression of k chain and gene arrenagement showed clonal rearrangement Of immunoglobulin genes, suggesting it had evolved into B cell lymphoma. Conclusins Castleman disease is a special giant lymph node hyperplasia, which can occur at any age. Differential disgnosis of it includes reactive follicular hyperplasia and lymphona, and special attention should be paid for cases with probable malignant transformation.