Castleman综合征合并抽搐1例伴文献分析  

Castleman Syndrome Complicated with Convulsions:A Case Report and Literature Analysis

作　　者：詹舒坤 翁斌[1,2,3] 王程毅 王世彪[1,2,3] ZHAN Shu-kun;WENG Bin;WANG Chen-yi;WANG shi-biao(College of Clinical Medicine for Obstetrics&Gynecology and Pediatrics,Fujian Medical University,Fujian Provincial Maternity and Children’s Hospital,Fuzhou 350005,Fujian Province,China;Fujian children’Hospital,Fuzhou 350001,Fujian Province,China;Fujian Branch of Shanghai Children’s Medical Center,Fuzhou 350001,Fujian Province,China)

机构地区：[1]福建医科大学附属妇儿临床医学院,福建省妇幼保健院,福建福州350005 [2]福建省儿童医院,福建福州350001 [3]上海交通大学附属上海儿童医学中心福建医院,福建福州350001

出　　处：《罕少疾病杂志》2022年第11期7-10,共4页Journal of Rare and Uncommon Diseases

摘　　要：目的 背景Castleman病(CD)在儿童中是罕见淋巴增生性疾病,预后差,近年来对CD的发病机制、诊断和治疗的研究较前增多。方法 加强对CD的认识,提高临床医师对该病的诊疗水平。方法 报道患儿于2015年开始反复于福建省妇幼保健院住院治疗,治疗效果不佳,后2021年9月17日至今期间患儿间断于福建省儿童医院住院治疗,最后一次住院期间出现抽搐3次,结合相关文献,总结其诊断及治疗特点。结果 本例患儿病初表现为反复高热、伴腹部不适,肝脾大、淋巴结肿大伴中度贫血、血小板升高、CRP升高,曾误诊为脓毒血症、肺含铁血黄素沉积症、淋巴瘤,首次住院时发现单纯疱疹病毒Ⅰ型及支原体感染,期间予反复抗感染、大剂量激素等冲击治疗,病情无明显好转。通过腹部彩超:肝门及胃底周边淋巴结肿大,肝脾肿大;2021年9月23日MRI回报肝左叶及肝Ⅵ段异常信号,以及胃底旁、肝胃间隙及肝门区多发肿大淋巴结。于2021年9月24日全麻下行腹腔肿物活检术+肝活检术+经纤维支气管镜肺泡灌洗术,病理回报符合Castleman病(混合型),最终诊断多发型castleman综合征。现予托珠单抗治疗后,病程中出现反复抽搐,排除其他疾病后目前考虑系castleman综合征引起抽搐。结论 CD在儿童中是一种较为罕见的疾病,进展迅速,预后较差,且明确诊断需行组织病理学检查,而针对IL-6通路靶向治疗可能成为CD患者有效治疗方案。Objective Background Castleman's disease(CD)is a rare lymphoproliferative disease in children with poor prognosis.The research on the pathogenesis,diagnosis and treatment of CD has increased in recent years.Methods To strengthen the understanding of CD and improve the diagnosis and treatment level of clinicians.Methods it was reported that the children were hospitalized in Fujian maternal and child health hospital repeatedly in 2015,and the treatment effect was poor.From September 17,2021 to now,the children were intermittently hospitalized in Fujian children's Hospital,and convulsions occurred three times during the last hospitalization.Combined with relevant literature,the diagnosis and treatment characteristics were summarized.Results The initial manifestations of this case were recurrent high fever with abdominal discomfort,hepatosplenomegaly,lymphadenopathy with moderate anemia,elevated platelet and CRP.It was misdiagnosed as sepsis,pulmonary hemosiderosis and lymphoma.Herpes simplex virus type I and mycoplasma infection were found in the first hospital.During the period of repeated anti infection and high-dose hormone shock treatment,the condition did not improve significantly.Through abdominal color Doppler ultrasound:enlargement of lymph nodes around hilar and gastric fundus,hepatosplenomegaly;On September 23,2021,MRI reported abnormal signals in the left lobe and VI segment of the liver,as well as multiple enlarged lymph nodes in the paragastric fundus,hepatogastric space and hilar area.Abdominal mass biopsy+liver biopsy+alveolar lavage via fiberoptic bronchoscope were performed under general anesthesia on September 24,2021.The pathological return was consistent with Castleman disease(mixed type),and the final diagnosis was multiple Castleman syndrome.After being treated with tozumab,there were repeated convulsions in the course of the disease.After excluding other diseases,it is considered to be caused by Castleman syndrome.Conclusion CD is a rare disease in children,with rapid progress and poor prognos

关 键 词：巨淋巴结增生 抽搐 CASTLEMAN病 诊断 治疗 病例报告 

分 类 号：R322.25[医药卫生—人体解剖和组织胚胎学]