摘要
目的探讨Castleman病（Castleman＇s disease,CD）的临床病理特点,通过对其免疫组化及基因重排结果的分析以提高对该病的认识。方法对12例Castleman病进行组织学、免疫组化和基因重排分析,着重探讨其免疫表型及克隆性基因重排的特点。结果 12例Castleman病患者中,男性4例,女性8例,年龄8~71岁。组织病理学分型：透明血管型（hyaline vasculartype,HVCD）4例,浆细胞型（plasma cell type,PCCD）5例,混合型（mixed cell type,Mixed CD）3例。HVCD表现为淋巴滤泡生发中心与淋巴窦大部分消失,滤泡间小动脉增生伴透明变性,且插入滤泡中心内;外套层淋巴细胞层次显著增多并呈同心圆状排列。PCCD的小动脉的透明变性和外套层同心圆状改变不明显,淋巴窦尚存,滤泡间有大量成熟浆细胞增生。混合型则兼有上述两者的改变。免疫组化结果显示,无论HVCD或PCCD,滤泡内均有CD21和CD68阳性的滤泡树状突细胞（FDC）增生,滤泡套细胞层内CD57的限制性表达,以及滤泡间组织细胞与吞噬细胞的增生。此外,血管内皮抗原CD31、CD34均弥漫表达,HVCD滤泡内的CD31、CD34阳性血管密度高于PCCD;PCCD滤泡间有大量CD138阳性的浆细胞,而在HVCD中几乎不表达。基因重排结果：12例中8例为Ig与TCR基因重排阴性,有4例局限型Castleman病患者基因重排呈阳性反应,其中1例为IgL重排阳性,1例为IgH和IgK基因重排阳性,1例为TCRD与TCRG基因重排阳性,1例为IgH和TCRB基因重排均阳性。结论除根据组织学特征之外,免疫组化染色有助于Castleman病的病理诊断和组织学分型,尤其对组织学特征不典型的病例可进行有效的鉴别诊断,免疫球蛋白基因和TCR基因重排分析可明确病变是否有克隆性增生或恶性转化。
Purpose To investigate the clinicopathological,immunophenotype and clonality of Castleman＇s disease（CD）.Methods Twelve cases of CD,including 8 females and 4 males with ages ranging from 8 to 74 years,were collected for immunohistochemistry and immunoglobulin（Ig） and T-cell receptor（TCR） gene rearrangement analysis.Results Among 12 cases,8 cases were localized type CD,and 4 cases were multicentric type CD.Histologically,4 cases were hyalinovascular variants（HVCD）,5 cases were plasma cell variants（PCCD）,and the other 3 cases were mixed type（mixed CD）.The involved lymph nodes consisted of numerous regressively transformed follicles,which showed marked layering of small lymphocytes in an ＂onion-skin＂ fashion,surrounding one or several central hyalinized vessels in HVCD,but were not obvious in PCCD.Instead,PCCD usually showed abundant plasma cells between the interfollicular areas.Immunohistochemically,HVCD and PCCD showed similar mixed B and T cell immunophenotypes,accompanied by CD31 and CD34 positive and proliferating vasculatures,CD68 and CD163 positive and proliferating interfollicular mononuclear/macrophagic cells,and CD21 and CD68 positive but CD35 negative follicular dendritic cells.In addition,PCCD was rich in CD138 positive plasma cells arranged in ＂sheet-like＂ pattern among the interfollicular areas,whereas in HVCD,CD138 were normally negative.Most cases（8/12） showed polyclonal B-and T-cell populations,as demonstrated by Ig heavy chain and TCR gene rearrangement analysis.However,two patients had a clonal rearrangement of the Ig genes,one was positive in the TCR gene rearrangement,and one had both IG and TCR gene clonal rearrangements.Conclusion Castleman＇s disease is typically a multiclonal hyperplasia disorder.Immunohistochemistry and gene rearrangement analysis can be used in the diagnosis and differential diagnosis of the disease.
出处
《临床与实验病理学杂志》
CAS
CSCD
北大核心
2011年第4期361-366,375,共7页
Chinese Journal of Clinical and Experimental Pathology
