自体干细胞移植治疗以Castleman病为表现的POEMS综合征1例报道并文献复习  

Autologous stem cell transplantation for the treatment of POEMS syndrome manifested by Castleman′s disease:a case report and literature review

作　　者：余浩源 惠卉 上官思雨 邱婷婷 李振宇 徐开林 李德鹏 YU Haoyuan;HUI Hui;SHANGGUAN Siyu;QIU Tingting;LI Zhenyu;XU Kailin;LI Depeng(Department of Hematology,the Affiliated Hospital of Xuzhou Medical University,Xuzhou,Jiangsu 221002,China)

机构地区：[1]徐州医科大学附属医院血液科,江苏徐州221002

出　　处：《徐州医科大学学报》2023年第9期689-692,共4页Journal of Xuzhou Medical University

摘　　要：目的研究以Castleman病为表现的POEMS综合征,提高对此类疾病诊治的认识。方法选取徐州医科大学附属医院血液科收治的1例Castleman病伴POEMS综合征患者,对其临床资料进行分析,并复习国内外相关文献。结果患者2015年6月因“淋巴结肿大半年”至徐州医科大学附属医院血液科门诊就诊。行颈部淋巴结穿刺活检病理诊断为Castleman病(浆细胞型)。PET/CT结果示:符合Castleman病表现,淋巴瘤不除外。于外院查血清M蛋白测定:IgAλ型M蛋白阳性血清游离轻链-λ(sFLC-λ)40.1 mg/L,sFLC-κ101.0 mg/L;可溶性蛋白电泳(SPE):M蛋白%4.5%,β-2微球蛋白10.1%,白蛋白51.7%;Ig3项:IgA 10.67 g/L;结合此前淋巴结活检结果及相关临床表进而诊断为POEMS综合征。于2018年11月行自体干细胞移植,移植后定期随访复查3年余,虽部分肿大淋巴结无法缓解,但无POEMS综合征相关症状,体检及实验室检查未见特殊异常。结论Castleman病及POEMS综合征均为罕见病,临床上容易误诊漏诊,同时罹患Castleman病与POEMS综合征的病例十分罕见,对于其中的关联、发病机制、预后影响因素仍不明了,有待进一步更深层次的研究。Objective To investigate polyneuropathy,organomegaly,endocrinopathy,monoclonal protein,and skin changes(POEMS)syndrome manifested by Castleman′s disease,in order to improve the understanding of the diagnosis and treatment of this disease.Methods One Castleman′s disease patient with POEMS syndrome who were admitted to Department of Hematology,the Affiliated Hospital of Xuzhou Medical University was selected to analyze clinical data.Furthermore,relevant literature at home and abroad was reviewed.Results The patient was admitted to Department of Hematology,the Affiliated Hospital of Xuzhou Medical University due to"swollen lymph nodes for half a year"on June 2015.Through aspiration biopsy of cervical lymph nodes,the patient was pathologically diagnosed with Castleman′s disease(plasma cell type).PET/CT results were consistent with the manifestation of Castleman′s disease,while lymphoma was not excluded.The serum M-protein was determined in another hospital and the results indicated that IgAλ-type M-protein positive serum free light chain(sFLC)-λwas 40.1 mg/L,sFLC-κwas 101.0 mg/L;soluble protein electrophoresis(SPE):M-protein%4.5%,β-2 microglobulin 10.1%,albumin 51.7%;Ig3 item:IgA 10.67 g/L.According to previous lymph node biopsy results and related clinical manifestations,the patient was diagnosed with POEMS syndrome.On November 2018,autologous stem cell transplantation was performed.After transplantation,the patient was regularly followed-up and re-examined for more than three years.Although some enlarged lymph nodes could not be relieved,there were no symptoms related to POEMS syndrome.No special abnormality was found in physical examination and laboratory results.Conclusions Both Castleman′s disease and POEMS syndrome are rare diseases that are easily misdiagnosed and missed in clinical practice.The cases of Castleman′s disease and POEMS syndrome at the same time are very rare.The association,pathogenesis and prognostic factors of Castleman′s disease and POEMS syndrome are still unknown and need

关 键 词：CASTLEMAN病 POEMS综合征 自体干细胞移植 诊断 

分 类 号：R551.2[医药卫生—血液循环系统疾病]