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[摘要]
?Castleman病(Castleman Disease,CD)又称巨大淋巴结增生,淋巴结错构瘤,良性巨淋巴瘤,血管滤泡淋巴组织增生,淋巴组织肿瘤样增生,是一种原因未明的反应性淋巴结病之一,临床较为少见。1956年Castleman等【1】正式报道一种局限于纵隔的肿瘤样肿块,组织学显示淋巴滤泡及毛细血管明显增生的疾病称为血管滤泡性淋巴结增生(vascular follicular lymphnode hyperplasia) ,1969年Flendring和Schillings【2】提出CD的另一形态学亚型,以浆细胞增生为特征,常伴全身症状。由于本病淋巴结肿大常十分明显,有时直径达10cm以上故又名巨大淋巴结增生(giant lymphnode hyperplasia)。由于多数CD患者的病理及临床表现均缺乏特异性改变,所以CD的诊断标准及分型十分必要。多中心Castleman病(MCD)与单中心Castleman病(UCD)存在显著差异,MCD常出现包括发热、乏力、胸腔积液、腹水等全身系统受累症状较典型,且受累淋巴结广泛,同时可出现肝脾肿大等。MCD主要包括两个亚类:人类疱疹病毒8(human herpesvirus 8,HHV8)相关的MCD【3,4】和特发性多中心Castleman病(idiopathic multicentric Castleman disease,iMCD),目前尚未发现与iMCD相关的病因【5】。确定CD的分型需进行淋巴结活检、形态学检查等完整的临床评估,且对诊断至关重要。本文主要介绍CD的诊断及治疗进展。
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[Abstract]
?Castleman Disease (CD), also known as giant lymph node hyperplasia, lymph node hamartoma, benign giant lymphoma, Tumor-like hyperplasia of lymphoid tissue, is a form of lymphadenopathy of unknown cause, which is rare in clinical. In 1956, Castleman al.【1】In 1969, Flendring and Schillings【2】proposed another subtype of CD featured with proliferation of plasma cells, accompanied by systematic symptoms. Multicentric Castleman disease (MCD)’s commonly reported systematic symptoms are fever, fatigue, polyserositis, hepatosplenomegaly and lymphnode diseases, which are distinct from unicentric Castleman disease (UCD). There are two kinds of MCD: human herpesvirus 8 (HHV8) associated multicentric Castleman’s disease (HHV8)【3,4】and idiopathic multicentric Castleman’s disease of which any definitely causes has not been found yet【5】.reported a disease with active hyperplasia of capillaries and lymphoid follicles named vascular follicular lymphnode hyperplasia.. Lymph node biopsy and morphological examination are crucial to classification and diagnosis. In this paper we will talk about the development of diagnosis and treatment of CD.
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