后腹膜巨淋巴细胞增殖症1例诊疗得失分析及文献复习  

A Case of CD's Analysis of Diagnosis and Treatment and Literature Review

作　　者：李宁[1] 陈国俊[1] 任海林[1] 张宝琳[1] 张海芳 LINing;CHEN Guo-jun;REN Hai-lin;ZHANG Bao-lin;ZHANG Hai-fang(Affiliated Hospital of Qinghai University,Xining,Qinghai,810001)

机构地区：[1]青海大学附属医院,青海西宁810001

出　　处：《智慧健康》2018年第10期89-91,97,共4页Smart Healthcare

摘　　要：巨淋巴细胞增殖症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,临床上罕见,易误诊。2016年10月,青海大学附属医院皮肤科收治1名初以"系统性红斑狼疮"为诊断的全身多发皮肤、粘膜溃烂的女性患者。治疗效果欠佳,经进一步检查和泌尿外科会诊确诊为后腹膜巨淋巴细胞增殖症引起的副肿瘤性天疱疮。行手术、对症支持治疗,康复出院,皮肤粘膜病变明显好转。术后病检:巨淋巴细胞增殖症。因本病罕见,皮肤科相关检查未能及时确诊,病理科亦未能对皮肤活检给出有积极意义的诊断,影像中心的CT检查对术前确诊本病起到重要的意义,术前、术中、术后均因患者经济原因未能应用丙种球蛋白,致使术后出现白细胞降低,咳嗽咳痰等肺部症状。但应用白蛋白替代治疗,效果较好。CD is abnormal proliferative disease of unknown etiology of lymph node, accompanied by changes in the skin and mucous membranes. It is clinically rare and easily misdiagnosed. Qinghai university hospital Department of Dermatology receive a female patient who was diagnosised Systemic lupus erythematosus. And she was multiple skin and mucous membranes. Because of poor treatment effect, to give further examination and Urology consultation. Making a definite diagnosis is CD caused PNP. Giving surgery, symptomatic support therapy, she was rehabilitation and discharge. Skin and mucous membrane lesions improved significantly. Postoperative pathological examination：CD. Department of Dermatology failed to diagnose in a timely manner, and Pathology also failed to give a positive diagnosis of skin biopsy, because this disease is unusual. CT plays an important role in the preoperative diagnosis of this disease. The preoperative and postoperative patients because of economic reasons failed to apply gamma globulin. It cause postoperative appearance decreasing of white blood cell, pulmonary symptoms such as cough and expectoration. But Albumin replacement therapy, the effect is better.

关 键 词：巨淋巴细胞增殖症 副肿瘤性天疱疮 

分 类 号：R551.2[医药卫生—血液循环系统疾病]