POEMS综合征的心脏表现

HeartManifestationofPOEMSSyndrome

MainPointCase1Case2DiscussionCase1患者,男,43岁,农民。因突发性胸骨后压榨性疼痛半年,伴四肢麻木无力3个月入院。患者于半年前因突发性胸骨后压榨性疼痛1h入本院,当时血清心肌酶谱明显升高,心电图显示V1～4呈QS型,ST段弓背向上抬高,T波倒置,符合AMI动态演变,诊断为“急性前间壁心肌梗死”,按AMI予以治疗治疗后病情明显好转,稳定出院。3个月前病人无明显诱因出现双下肢麻木,继而双上肢麻木,并伴四肢无力、疼痛、行走困难,双手不能持物,腹胀,面部及双下肢水肿。当地医院就诊,腹部B超提示肝、脾肿大,大量腹水。经扩张冠状动脉、减轻心脏负荷、强心、利尿等治疗,腹胀、面部及双下肢水肿减轻,但四肢麻木、无力、疼痛较前明显加重,不能独自行走,。患者既往身体健康,家族中无类似病史,详询病史,患者配偶诉病人有阳痿史近1年。体格检查血压100/80mmHg体形消瘦,全身皮肤变黑、多毛,以四肢为著。

乳晕色深,乳腺增大、有触痛。心界向左下轻度扩大,听诊心音低钝、可闻及心尖部Ⅱ级收缩期杂音。肝脏在右侧肋下4cm可触及,脾在左侧肋下4cm可触及，移动性浊音阴性。双下肢无水肿。眼底检查视乳头边缘不清。神经系统检查双肘以下及双膝以下深、浅感觉减退,双侧腓肠肌压痛明显,四肢肌张力低下、肌肉轻度萎缩,双上肢远端肌力2级、近端4级、双下肢远端肌力0级、近端3级、双足部下垂,病理反射未引出。辅助检查血常规基本正常，尿常规:红细胞少许、白细胞少许、蛋白(＋＋)、管型2～3/HP血清生化：血清肌酐144μmol/L、尿素氮13.8mmol/L、尿酸455μmol/L、血钠131mmol/L、血钾4.8mmol/L、血钙2.54mmol/L、血磷1.23mmol/L,血糖5.2mmol/L。免疫指标阴性骨髓穿刺:三系明显增生,红细胞系统尤为明显,浆细胞占0.202。骨髓活组织检查(第5腰椎)示浆细胞骨髓瘤。心电图:窦性心律、左前半分支阻滞、陈旧性前间壁心肌梗死。腹部B超:肝、脾、双肾明显增大。心脏UCG:见前壁近心尖处1.7cm一段心室壁变薄、厚度7mm、活动度差,左心室射血分数0.72,左心房内径34mm,左心室内径55mm,升主动脉内径23mm,各瓣膜正常。全身核素骨扫描:除第5腰椎外未发现异常浓染区。治疗治疗经过:入院第6日早晨,患者突然出现右侧肢体偏瘫,完全性运动性失语,饮水呛咳。体格检查:神志清晰,失语,右侧上肢和下肢肌张力低下、肌力0级,右侧巴宾斯基征阳性。颅脑CT检查提示左侧大脑梗死。予以脱水、溶解血栓、营养神经治疗,病情未见好转,于当日14:00许出现高热、烦躁、无尿、极度呼吸困难,咯粉红色泡沫样痰。查脉搏细弱,血压80/40mmHg,双肺满布哮鸣音及湿啰音,心率140～150次/分、心音听不清(因双肺哮鸣音掩盖)。立即予以升压、强心、利尿、减轻心脏负荷及对症治疗,病情无好转,抢救无效,患者于当日22:00死亡。CaseReport2ChiefComplaint：64-year-oldwomanwasadmittedtohospitalbecauseofsystemicedemaandexertionaldyspneaPresentHistoryShehadbeendiagnosedashavinghypertensionattheageof60forwhichshewastreatedelsewherewithacalciumantagonist.Shehadexperiencedprogressiveedemafortheprevioussixmonthsandamonthbeforeadmissionhaddevelopedexertionaldyspnea.PastHistoryShehadnopersonalorfamilyhistoryofanyheartdiseases.PhysicalExaminationHeight,153cm;bodyweight,63kg;T37.5°C;BP,160/80mmHg;andapulserate,80beats/minandregular.Severepittingedemainthedistalextremitiesanddiffusehyperpigmentationoftheskin.Thejugularveinsweredistended.Asystolicmurmurwasaudibleatthefourthintercostalspaceneartheleftsternalborder,andmoistraleswereaudibleinthebilaterallungfields.Theabdomenwasdistended.Neurologicalexaminationrevealedmuscleweaknessandgloveandstockingtypedysesthesiainthedistalextremities.Patellarandachillestendonreflexeswereabsent.Laboratorydata

BloodRoutine:RBC4.41×1012/L,Hb12.8g/L,Hct38.5%;WBC7.4×109/L,N74%;PLT560×109/LCRP:0.1mg/dlBloodChemistry:TP6.4g/L,Alb55.4%,BUN12mg/dl,Cr0.6mg/dl,AST8U/L,ALT5U/L,LDH259U/L,ALP216U/L,TC112mg/dl,triglyceride100mg/dlSerumelectrolytesandurinewerenormal.LaboratorydataBloodsugar104mg/dl,HbA1C4.9%Arterialbloodgasanalysis:pH7.409;PO261.9mmHg;PCO240.3mmHgAbdominalCTshowedsplenomegalyandmildascites.Anelectrocardiogramwasnormal,exceptforflatTwaves.ChestX-rayCardiomegaly(cardiothoracicratio=56%)MildpulmonarycongestionAndpleuraleffusionEchocardiogramLeftventricularhypertrophyanddilatationwithnormalcontractility(ejectionfraction=76%),leftatrialdilatation,andmildpericardialeffusionTreatmentThepatientwasdiagnosedashavinghigh-outputheartfailureandwastreatedwithdiuretics(Torasemide8mg/day).HerhypertensionwithleftventricularhypertrophywastreatedwithanangiotensinIIreceptorantagonist(Losartan50mg/day).Threeweeksafteradmission,thepatient'sheartfailuresymptomsdisappeared.Butahighcardiacoutputandadecreasedsystemicvascularresistancepersisted.Coronaryangiographywasnormal,andaleftventriculogramshowedleftventriculardilatationwithnormalcontractility(ejectionfraction=67%)What’sgoingonAfterimprovementofherheartfailure,thepatient'smuscleweaknessanddysesthesiainthedistalextremitiesworsened.Hernerveconductionvelocitieswereseverelydelayed.SerumimmunoelectrophoresisrevealedanM-proteincomponentoftheIgA-λ,type.Peripheralneuropathy,papilledema,splenomegaly,skinhyperpigmentation,andsystemicedema.ShewasreferredtoanotherhospitalforfurtherexaminationandtreatmentofherCrow-Fukasesyndrome.DiscussionDiscussionPOEMSsyndromeisaplasmacelldyscrasiaassociatedwithpolyneuropathy,organomegaly,endocrinopathy,M-protein,andskinchanges.Pathogenesis浆细胞病关系密切:浆细胞分泌的毒性物质作用于全身各脏器,导致多系统损害。细胞因子自身免疫病毒作用Diagnosis①多发性周围神经病:四肢渐进性弛缓性瘫痪,对称性进行性感觉障碍呈手套袜套样感觉减退或痛觉过敏。②脏器肿大:以肝、脾和淋巴结肿大多见。③内分泌障碍:阳萎、闭经、糖尿病、甲状腺功能减低。④M蛋白血症:多为IgG,λ轻链。⑤皮肤改变:色素沉着、皮肤增厚、多毛。⑥水肿、胸水、腹水。⑦视乳头水肿,脑脊液蛋白细胞分离现象,低热,多汗。典型病例具备前5项即可诊断。不典型病例必须具有第1和第4项,再加上第2、3、5项中的任1项也可诊断。第6、7项作为诊断参考。Treatment采用免疫抑制和细胞毒性药物,控制浆细胞病后可使症状减轻,但难于根治。Prognosis首发症状起,存活时间为6个月至7年,平均33个月,个别患者可生存13年。Aboutonethirdofpatientswiththissyndromedieasaresultofheartfailure.POEMS综合征常以心梗、心衰、肝脾大、水肿为突出表现，进而掩盖了其他多系统慢性病变。对POEMS综合症的认识不足，常常影响原发病的早期诊断及治疗。因此，对出现心脏以外多系统慢性进行性病变患者应高度怀疑POEMS综合征，进一步做相关检查确诊。HypothesisAcaseofPOEMSsyndromethatwasassociatedwithpulmonaryhypertensionsuggestedthattherewasaclosecorrelationbetweenserumVEGF(vascularendothelialgrowthfactor)levelsandpulmonaryarterypressures.Inthepresentcase,theserumVEGFlevelwasmarkedlyelevatedafterrecoveryfromheartfailure.wesuspectthatVEGFmayhavedecreasedsystemicvascularresistanceinourpatientresultingint