Castleman病变异型POEMS综合征1例报告并文献复习  

CASTLEMAN DISEASE VARIANT OF POEMS SYNDROME:A CASE REPORT AND LITERATURE REVIEW

作　　者：李昱昕 许翔[2] 张晓林 邢宝迪 赵文娟[1] 郝大鹏[4] 刘斌[5] 陈云庆[6] 董冰子 王忠超[1] 孙晓方[1] 赵宇航[1] LI Yuxin;XU Xiang;ZHANG Xiaolin;XING Baodi;ZHAO Wenjuan;HAO Dapeng;LIU Bin;CHEN Yunqing;DONG Bingzi;WANG Zhongchao;SUN Xiaofang;ZHAO Yuhang(Department of Endocrinology and Metabolism Diseases,The Affiliated Hospital of Qingdao University,Qingdao 266003,China)

机构地区：[1]青岛大学附属医院内分泌与代谢性疾病科,山东青岛266003 [2]青岛大学附属医院国际医学部,山东青岛266003 [3]青岛大学附属医院人力资源部,山东青岛266003 [4]青岛大学附属医院放射科,山东青岛266003 [5]青岛大学附属医院风湿免疫科,山东青岛266003 [6]青岛大学附属医院病理科,山东青岛266003

出　　处：《精准医学杂志》2023年第6期542-545,549,共5页Journal of Precision Medicine

基　　金：国家自然科学基金青年项目(81800769)。

摘　　要：目的提高临床医师对POEMS综合征的认识,提高不典型POEMS综合征的临床诊治经验。方法回顾性分析2020年9月于我院内分泌科住院的1例Castleman病(Castleman disease,CD)变异型POEMS综合征患者的临床资料,并进行文献复习。结果本例患者以内分泌异常(性腺功能减退)为首发表现,继而出现皮肤损害、神经源性损害、多发浆膜腔积液、多发淋巴结肿大等表现,临床上符合POEMS综合征诊断,但血液学检查无克隆性浆细胞证据。后经多学科会诊,完善淋巴结活检,病理检查提示为CD,且血浆中血管内皮生长因子(VEGF)水平升高,最终诊断为CD变异型POEMS综合征。结论临床医师在诊治POEMS综合征时需具备多元思维,当临床上高度怀疑POEMS综合征但不完全符合强制诊断标准时,应积极寻找相关证据,尤其需重视病变部位活检和血浆VEGF水平在该病中的诊断价值,以减少漏诊、误诊。Objective To improve the awareness of POEMS syndrome among clinicians,and to increase the experience in the clinical diagnosis and treatment of atypical POEMS syndrome.Methods A retrospective analysis was performed for the cli-nical data of a patient with Castleman disease variant of POEMS syndrome who were hospitalized in Department of Endocrinology in our hospital in September 2020,and a literature review was performed.Results The patient had the initial manifestation of endocrine abnormality(hypogonadism),followed by other manifestations such as skin damage,neurogenic damage,multiple serous effusion,and multiple lymph node enlargement,which were consistent with the diagnostic criteria for POEMS syndrome,but hematological examination showed no evidence of clonal plasma cells.After multidisciplinary consultation and lymph node biopsy,pathological examination suggested Castleman disease,with an increase in the level of vascular endothelial growth factor(VEGF)in plasma,and finally the patient was diagnosed with Castleman disease variant of POEMS syndrome.Conclusion Clinicians need to have pluralistic thinking in the diagnosis and treatment of POEMS syndrome,and when POEMS syndrome is highly suspected in clinical practice without fully meeting necessary diagnostic criteria,they should actively look for relevant evidence and emphasize the value of lesion biopsy and plasma VEGF level in the diagnosis of this disease,so as to reduce missed diagnosis and misdiagnosis.

关 键 词：POEMS综合征 CASTLEMAN病 性腺功能减退症 血管内皮生长因子类 病例报告 

分 类 号：R584.2[医药卫生—内分泌] R557.4[医药卫生—内科学]