后腹膜巨淋巴细胞增殖症合并副肿瘤性天疱疮的诊疗策略

摘要

巨淋巴细胞增殖症(CD)是一种病因未明的淋巴结异常增殖性疾病,伴发皮肤、粘膜改变,称为副肿瘤性天疱疮(PNP),发生于后腹膜者临床上较罕见,临床表现无特异性,易误诊,确诊需要病理学检查,并根据其分型不同采取不同的治疗方式.本文针对其特点及诊疗策略进行述评,以提高对巨淋巴细胞增殖症的认识及诊治策略.%Giant lymphocyte proliferation disease called paraneoplastic pemphigus is an abnormal lymphoproliferative disorder of unknown etiology,concomitant changes in skin and mucous membranes.Giant lymphocyte proliferation disease rarely occurs in the retroperitoneum.Its clinical features are not special and easily misdiagnosed.Different treatment modalities should be adopted according to their pathological classification.This article summarized its characteristics,diagnosis and treatment strategy,in order to improve the understanding and diagnosis and treatment level of giant lymphocyte hyperplasia