摘要目的 研究Cagtleman病(CD)的临床及病理类型与各种合并症的关系,推测各种合并症的发病机制,以提高对本病的认识.方法 回顾性分析北京大学第一医院自1977年以来共53例CD患者的临床特点,同时分析临床合并症与临床类型和病理类犁的关系.结果 53例CD患者中单中心型32例(60.4%),多中心型21例(39.6%);病理分型示透明血管型(HV)37例(69.8%),浆细胞型(PC)9例(17.0%),混合型(MIX)7例(13.2%).患者中32例有合并症,占60.4%.根据受累器官不同分为累及皮肤、内脏和血液系统.临床类型与某些合并症的发生密切相关,32例单中心型的主要合并症为副肿瘤性天疱疮(PNP)和闭塞性细支气管炎(BO),21例多中心型的主要合并症为肾脏、血液改变.病理类型与合并症的发生密切相关:透明血管型主要合并症为PNP和BO,浆细胞型和混合细胞型主要合并症为内脏和血液受累.合并PNP的CD患者的临床和病理分型均不同于其他CD.Kaplan-Meier分析显示:伴有合并症的患者生存率明显低于无合并症的患者(P=0.028).结论 CD患者的合并症与其临床、病理分型有关,合并PNP的CD患者应单独分型,有无合并症是影响CD患者预后的关键因素.

abstractsObjectives To investigate the clinical and pathological subtypes of Cagtlenum's disease (CD)and their relationship with complications.Methods The clinical complications of 53 patients with CD and the relationship of these complications with clinical and pathological subtypes were analyzed retrospectively.Results Among 53 CD patients,32(60.4%)were classified as uni-centric type and 21(39.6%)multicentrie type.Histopathological examination showed that 37 cases(69.8%)were hyaline vascular variants (HV),9(17.0%)plasmacytic variants(PC),and 7(13.2%)mixed cellular variants(Mix).Cornlplications were identified in 32(60.4%)patients,including the involvements of skin,internal organs and hematopoietic system.Some complications were closely associated with the clinical subtype of CD:the majority of complications in the 32 uni-centric CDs were paraneoplagtic pemphigus(PNP)and bronchiolitis obliterans (BO),and those in 21 multi-centric CDs were the involvements of kidney and hematopoietic system.The complications were different among the three kinds of histopathological subtypes:PNP and BO were the predominant complications of HV variants.while the internal organ and hematopoietic system involvements were those of PC and Mix variants.The clinical and histopathoiogical classification of CD patients with PNP were different obviously from other subtypes of CDs.In Kaplan-Meier survival analysis,the survival rate of those with complications wag significantly lower than those without complication(P=0.028).Conclusion The clinical complications of CDs are related to their clinical and histopathological subtypes.CD patients with PNP should be considered as a unique entity to tailor the therapy.The presence of clinical complications is an independent prognostic factor in CD patients.