Yu Hang, Yao Fang, Li Yue, Li Jian, Cui Quan-Cai
Department of Gastroenterology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences Beijing 100730, China.
Department of Hematology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences Beijing 100730, China.
Int J Clin Exp Pathol. 2015 Oct 1;8(10):13578-83. eCollection 2015.
POEMS syndrome is a rare hematological disorder associated with plasma cell dyscrasia characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. Castleman disease is a lymphoproliferative disorder that can be present in POEMS patients, which can be defined as Castleman disease variant of POEMS syndrome. Herein, we described a 24-year-old male patient diagnosed with this syndrome and also suffered from multiple cerebral infarctions. This patient showed no evidence of monoclonal gammopathy and failed to have electromyography examined. The final diagnosis was established with the help of the axillary lymph node biopsy. As a rare case of POEMS syndrome without evidence fulfilling the major mandatory diagnostic criteria and with cerebrovascular involvement, its characteristics was discussed with a brief literature review in order to facilitate further understanding of the POEMS syndrome.
POEMS综合征是一种罕见的血液系统疾病,与浆细胞异常增生有关,其特征为多发性神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变。Castleman病是一种淋巴增殖性疾病,可出现在POEMS综合征患者中,可定义为POEMS综合征的Castleman病变异型。在此,我们描述了一名24岁男性患者,他被诊断患有该综合征,同时还患有多发性脑梗死。该患者未显示单克隆丙种球蛋白病的证据,也未进行肌电图检查。最终诊断是在腋窝淋巴结活检的帮助下确定的。作为一例罕见的POEMS综合征病例,缺乏符合主要强制性诊断标准的证据且伴有脑血管受累,我们通过简要的文献回顾对其特征进行了讨论,以便于进一步了解POEMS综合征。