26例系统性淀粉样变性患者的临床分析

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1、苏州大学硕士学位论文26例系统性淀粉样变性患者的临床分析姓名：陆静娟中请学位级别：硕士专业：临床医学指导教师：柴华旗20110526例系统性淀粉样变性患者的临床分析中文摘要0的皿鮒彩疑统储谴粉揪鼬姗如綢碱点提攜祈临梯系紂麻檢拝变性的认识。方湖顽H性俞拥我阶轴()逾湘至2011年2月来26例经组织活检和临床证实符令钿f憫掰'繚苗幽撷綁綸拡点实棘瀚鑼瀚绽側幽淪娩情况。结果系统性淀粉样变杵.26例,男性10例,女性16例。发摘年龄从42〜79岁，中位年龄60.5^e26例患者均绊活检病理确诊为淀粉样变

2、性，M例患者经免疫组化证实存在克隆性免疫球蛋门轻链，其中5例患呂确诊为训继发性淀粉样变性，具余12例患者*进行免疫组化检査。该组患者中仔脏16例(61.5%).心脏10例(38.5%)、消化系綁例(30.、8楡至熬竝乗统灘觸则狡录蕭他的翻谿㈱翩淌怖嫁及多个器官系绒J翻删g極现为川明駅麻的乏力、体葡邢释、协肿、人魅礦蚩松尿四丿阙«瞼他別嘶删麟勢物弼制单醴删删桝iIM幽链曲先汾描掠腹惯淞粧憫样变Iff挪购J请/淞,躺倆慚I核酬m送联联泼雄淞淞繚仍诚贩顾薄氷㈱锵松治瓠葡眦1细列趣肘楝。，例?例怨堆駆挪

3、剜娜彭呦死砌g熾叫能裒竭。结ST淞卿性淀糊齣如弧摘鄰硝做聯撒域碌裁胭阑蹦璇秘病淀杭变为淀粉解物鄭㈱W細伺帧删致o2.该病可累及多器官多系统，引起受累器官结构改变和功能障碍。址常见受累器官为心脏、肾脏。其临床表现多种多样，误诊率髙，预后较至。3•系统性淀粉样变性无创件常规实验室检查无明!上特异性，超声心动阁、免疫蛋白电咖II绷有嚨翱®助但働确諭需靠綢頻检c关键词刼瞬㈱懈械扛岬检:临床特征作<:稱饑絹指导教师：柴华旗A^nicalanalysisof26casesofsystemicamyloido

4、sisAbstractObjectiveffosummarizetheclinicalcharacteristicsofsystemicamyloidosisinourhospitalinorderXoimproveitsdiagnosislevel.MethodsThedimcal/dataof26©agentswithsystemicamyloidosis,admittedtoourhospitalsince2002XofiOlLwerefiAialvzed.ResultsTherewere

5、26patientsWithsystemicamyloidosisJi)casesAfteramen，16AAAAAAAAAAAcaseswerewomen.Amongthe£6幽側aa]4patientsdidthe谕糊袖istochemistrythatdemonstratedtheexistence£>fM6Moclonalimmunoglobulinlightchain.5wereWUrttiple询flffi®bassocia〔edamyloidosis.The斛鬼Msthatmost

6、曲細enilyaffectedAnthesepatientswerethekidneys(6L5%)^heart(38.5%)>digestive硼^^(轴8%)andnervoussystem(23」％),somepatients)werefound/withmultipleorgan械瞅.ThemainjctowiiA&ymptomswerefoundXobeunexplained矗隔,abundantproteinuria,limbnumbness.Usingantitumordrugs甜

7、chasablationagentXosuppressthe砂碱clonalM艮顾acellproliferationandreducetheproductionaflightchainsisthemaintreatmentofprimaryamyloidosis.TheMAAAcommontreatmentsofthe26patientsMereA1P(melphalan.prednisone)regimeorvelcadeusedincombinationA^jUAAtoAArnethaso

8、ne.5patientsdiedduringhospitalizationbecauseofheartfailure.©Mdusion1.systemicamyloidosisisa細'战砸箱俑amyloiddepositindilierentorgansleadingxomanifestations.2.1t^allyinxolvesmultipleorgansand吐妣融Heartand劇neyarethemost^q^ientlyinvolvedorgans.The聊馭卑gn&sedrat