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1、萬方數(shù)據(jù)中國醫(yī)學(xué)科學(xué)院學(xué)報ACTAACADEMIAEMEDICINAESINICAECastleman病的發(fā)病機制和治療進展韓瀟,周道斌中國醫(yī)學(xué)科學(xué)院北京協(xié)和醫(yī)學(xué)院北京協(xié)和醫(yī)院血液內(nèi)科,北京100730通信作者:周道斌電話:010—65295024,電子郵件:daobinzhou@yahoo.con·綜述·摘要:Castleman病(cD)是一種少見的淋巴組織增殖性疾病,病因可能與病毒感染、細胞因子調(diào)節(jié)異常和血管增生等有關(guān)。近年研究發(fā)現(xiàn),人類皰疹病毒(HHV)一8感染和白細胞介素(IL)一6過度表達在CD的發(fā)病機制中發(fā)揮重要作用。治療方法包括手術(shù)切除、放療、化療、抗病毒治療和靶
2、向治療等。多中心型CD目前尚無標(biāo)準(zhǔn)治療方法,總體療效欠佳。新式治療中抗HHV一8病毒治療效果不明確,抗CD20單抗和抗IL-6受體單抗已有良好療效的報道,沙利度胺和硼替佐米等也初見療效。關(guān)鍵詞:Castleman??;病因;治療;人類皰疹病毒一8;白細胞介素一6;利妥昔單抗;tocilizumab中圖分類號:R557+.4文獻標(biāo)識碼:A文章編號:1000_503X(2009)05—0639—05DOI:10.3881/j.issn.1000-503X.2009.05.029AdvancesinEtiologyandManagementofCastleman,SDiseaseHAN
3、Xiao,ZHOUDao-binDepartmentofHematology,PUMCHospital,CAMSandPUMC,Beijing100730,ChinaCorrespondingauthor:ZHOUDao—binTel:010—65295024,E—mail:daobinzhou@yahoo.conABSTRACT:Castleman’Sdisease(CD)isarare1ymphopr01iferatiVedisorder.TheetiologyofCDmayinvolveviralinfection,abnormalmodulationofcytokine
4、s,andangiogenesis.Humanherpesvirus(HHV)-8infectionandinterleukin-6(IL-6)overexpressionmayplaykeyrolesinthedevelopmentofCD.Treatmentop-‘‘ncludegical一,radiationtherapy,chemotherapj,antiviraltherapy,andgetedtherapy.Notlonsincludesurgicalexcisionradiationtherapychemotherapyantiviraltherapyandtar
5、getedtherapy●standardizedtreatmenthasbeenestablishedformulticentricCDandthetreatmentefficacyusuallyispoor.Amongnewlyavailableagents,theeffectivenessofantiviraltherapyagainstHHV-8isunclear;anti-CD20andanti-IL-6receptormonoclonalantibodieshaveshownpromisingefficacy;thalidomideandbortezomibhave
6、showntheirinitialefficacy.Keywords:Castleman’Sdisease;etiology;treatment;humanherpesvirus一8;interleukin一6;rituximab;tocilizumabCastleman病(Castleman’Sdisease,少見的淋巴增殖性疾病增生或者巨大淋巴結(jié)病布和器官受累情況CD)是一種,又名血管濾泡性淋巴組織,臨床上根據(jù)腫大淋巴結(jié)分分為單中心型(unicentricCastleman’Sdisease,UCD)和多中心型(multicentricCastleman’Sdisease,
7、MCD),病理可分為透明血管型(hyalinevascularvariant,HVV)、漿細胞型(plasmaActaAcadMedSin,2009,31(5):639—643cellvariant,PCV)和混合型。CD發(fā)病機制目前尚不十分清楚,近年來人類免疫缺陷病毒(humanimmu.nodeficiencyvirus,HIV)和人類皰疹病毒(humanherpesvirus,HHV)-8與CD的關(guān)系MCD的治療也已成為研究的焦點。越來越得到重視。V01.31No.5639萬方數(shù)據(jù)中國