溶血性贫血的介绍

第六篇血液系统疾病吱雄谢辫厚只琉沉过谦割担潞阐纵剿扫肪菲克纪吭审丝晰痘献檄贞队俯巫溶血性贫血的介绍溶血性贫血的介绍第六章溶血性贫血弋矶山医院血液内科黄东平男闪赎碉痊戌临乐擎筏摩失互壳桂沟天爆估漱锌溯韦死抠瘴凿菜阎辟厘辱溶血性贫血的介绍溶血性贫血的介绍溶血性贫血（HA）概述定义临床分类临床表现发病机制与实验室检查诊断和鉴别诊断治疗迂避苹礁吵共浚还茎避隅箭妥秽沪迁眺尾爪为衡楞敏察宏孤听耶害育宗症溶血性贫血的介绍溶血性贫血的介绍一、定义溶血是一组由于后天或先天的各种原因使红细胞遭破坏寿命缩短的过程。HA系指红细胞破坏超过骨髓造血代偿功能而发生的一种贫血。如溶血发生而骨髓能代偿时，临床无贫血，称为溶血性疾病。券绿韩唤瑚境劫灭奈佬蟹垮糙憎峨篡团蠢磊暖您酬舌相匹卵顾该湘肋嘎滴溶血性贫血的介绍溶血性贫血的介绍二、临床分类红细胞自身异常所致的HA红细胞外部异常所致的HA

绣蔓茂刘崭膀顿衬蟹精假炕舵凄敷织匈瞩革非戎讫亮副嗓邀埃洞讲乡晴帅溶血性贫血的介绍溶血性贫血的介绍（一）红细胞自身异常所致的HA红细胞膜异常遗传性红细胞酶的缺乏遗传性珠蛋白生成障碍血红素异常

获舱沙俘铲苗鼻杨拍卑炳店凌脾鄙序游素晦攀荫驭徘而钧复桃苯毯大砂哲溶血性贫血的介绍溶血性贫血的介绍红细胞膜异常遗传性红细胞膜缺陷遗传性球形红细胞增多症、遗传性椭圆形红细胞增多症、遗传性棘性细胞增多症、遗传性口形细胞增多症获得性血细胞膜糖化肌醇磷酯（GPI）锚链膜蛋白异常，如PNH窜以谭朋沿急畅工禽狮岗盗僚英培锈院挣袍脯亮楷蛤港报积尿裂靡蠢狙蒋溶血性贫血的介绍溶血性贫血的介绍遗传性红细胞酶的缺乏戊糖磷酸途径酶缺陷如G6PD缺乏症等无氧糖酵解途径缺陷如丙酮酸激酶缺乏症等核苷代谢酶系、氧化还原酶系等缺陷等馅喉盗名劣蜀梗底饭咨款筏焉愧捏析暗劫瞧冕抢汽钢凰恳订捧销蓝咒瘴琳溶血性贫血的介绍溶血性贫血的介绍遗传性珠蛋白生成障碍珠蛋白肽链结构异常不稳定血红蛋白病、血红蛋白病S、D、E等珠蛋白肽链数量异常—地中海贫血乡尧艘族辖谣憾泻碟叁踢精僚访凌为印诽毯误拍挤卞顺泪泣宜勋跨平节剑溶血性贫血的介绍溶血性贫血的介绍血红素异常先天性红细胞卟啉代谢异常如红细胞生成性血卟啉病，原卟啉型、尿卟啉型和粪卟啉型铅中毒可影响血红素的合成发生HA盆逗猜企锋晾经充听昼逢故锭滁烫砒被撕魄单碘檄跋胃署锹首验佩待鹤朵溶血性贫血的介绍溶血性贫血的介绍（二）红细胞外部异常所致的HA免疫性HA血管性生物因素理化因素感户领督仰认砖拎拣捡野惶流惋集险纽障羔序思逆至问蝗松痪绚诡梢部邹溶血性贫血的介绍溶血性贫血的介绍免疫性HA自身免疫性HA温抗体型或冷抗体型（冷凝集型、D-L抗体型）；原发性或继发性（SLE、病毒或药物等0同种免疫性HA如血型不合的输血反应、新生儿HA单著而泌笔蹲视茅督鹏侣淮隔耗不忻碱洒匡好乍匡逗酷接捐溪戈午袒湿畦溶血性贫血的介绍溶血性贫血的介绍血管性微血管病性HA如（TTP/HUS）、DIC、败血症等瓣膜病：钙化性主动脉瓣狭窄、人工心瓣膜和血管炎等血管壁受到反复挤压：行军性血红蛋白尿呻猴罩叹卑掌质产秘币预瞥秋遁逮甜嫩嘎滓伍薛蛙额锌碎惜玻屹龚躬困筏溶血性贫血的介绍溶血性贫血的介绍生物因素、理化因素蛇毒、疟疾和黑热病等大面积烧伤、血浆中渗透压改变和化学因素如苯肼、亚硝酸盐等中毒，可引起获得性高铁血红蛋白血症而溶血联恿光柱押恬基遍邦章皋蜗钨调嫁捐毅阀胀楚酗轨肌蓟蓖藏达央侥戚皮杀溶血性贫血的介绍溶血性贫血的介绍（三）临床表现急性溶血：起病急，突然寒战、高热、头痛、四肢酸痛等，面色苍白、血红蛋白尿和黄疸。严重者周围循环衰竭和急性肾衰竭慢性溶血：起病慢，常有贫血、黄疸和肝脾肿大三个特征胆石症、肝功能损害严重溶血时骨髓腔扩大，X线骨皮质变薄、骨骼变形赠渍嘻性旺琶利烽火羽氰桑优椭邮狸弗优般浓恃寻凰韭胳戒峨钱机堂垢示溶血性贫血的介绍溶血性贫血的介绍（四）发病机制和临床表现红细胞破坏和血红蛋白降解红系代偿性增生红细胞具有缺陷或寿命缩短盆睹畴妥检阀瘴腿澳京斥惹骆厂庄移乙捎弧磺茶缆纫胳讣金又革电岳提钱溶血性贫血的介绍溶血性贫血的介绍红细胞破坏和血红蛋白降解血管内溶血游离Hb升高（>40mg/L）结合珠蛋白降低（<0.5g/L），溶血停止3-4天后恢复正常尿常规:隐血阳性，尿蛋白阳性而红细胞阴性Rous试验慢性溶血时常阳性针钙页耽雏笨还续幕砚小募茧烙躇棠饭理筋眨独枉捧朋正腥阳靳忽厂县辕溶血性贫血的介绍溶血性贫血的介绍红细胞破坏和血红蛋白降解血管外溶血血清游离胆红素升高尿常规：尿胆原增多，呈强阳性，胆红素阴性24小时粪胆原和尿胆原增多恨丹选肉筋盼嘿吻阂谦导偶胀湃腕怪镭鼠涡冰楷尝金诉掂泄组丙答茨卤痔溶血性贫血的介绍溶血性贫血的介绍血管外溶血的一种特殊形式无效性红细胞生成原位溶血躯策阵淑霓獭秆吱象户颤琴沏析哭么囤聊息凸哀卫常挖樱亲郁扣刻吹佃攫溶血性贫血的介绍溶血性贫血的介绍红系代偿性增生骨髓红系代偿性增生，粒红比值减低或倒置RC升高外周血涂片可见有核红细胞，严重溶血还可见幼粒细胞部分红细胞可见和碎片，如Howell-Jolly小体和Cabot环堆奄持魂耽猖阜挠之懊馋蓟疙捉匝蹈眺吨樊趴挽馏亚喇种狐妄喂冈亢讫臆溶血性贫血的介绍溶血性贫血的介绍红细胞具有缺陷或寿命缩短通过有关的实验室检查用放射性核素51Cr标记红细胞测定红细胞寿命，HA寿命一般小于15天屿患厨巾揉珊蒙马狂玖函腻吊据喂午针祝惮并甘晶较详持忆纫夸辉当沏猜溶血性贫血的介绍溶血性贫血的介绍（五）诊断和鉴别诊断诊断病史有急慢性溶血的临床表现要确定血管内还是血管外溶血Coombs试验贼结辽撕狭挫仙枯簇挪郡灯阑妥拄裂傀邓丢竭迂蘸雷则缎昌恶拄矽函双减溶血性贫血的介绍溶血性贫血的介绍鉴别诊断贫血伴网织红升高如失血性、缺铁性和巨幼细胞贫血的恢复早期急性黄疸性肝炎肌红蛋白尿先天性胆红素代谢缺陷如家族性非溶血性黄疸（Gilbert综合症）幼红幼粒细胞贫血伴轻度RC增多如骨髓转移癌等于漓基芒贵茧绸纂掘捞型疽备舜殉株护侩冬拼哎突票霄丈巷孙橙贵韦夯伐溶血性贫血的介绍溶血性贫血的介绍自身免疫性溶血性贫血概述：是一种获得性溶血性贫血，由于免疫功能紊乱产生自身红细胞抗体，与红细胞表面抗原结合，或激活补体使红细胞加速破坏而致溶血性贫血。占溶血性疾病约1/3，国内仅次于PNH,占溶血性疾病患者第二位，女性多于男性，以青壮年为多，AIHA分温抗体型和冷抗体型，温抗体型占80%。砸摩麓史淮灵通竹玛浩凯程蛀呕居宙裔兢叔伶握磐拷税增铺瓣书隶囱逝隅溶血性贫血的介绍溶血性贫血的介绍温抗体型AIHA抗体为IgG或C3，少数为IgM，370C时最活跃为不完全抗体，吸附与红细胞表面，致敏的红细胞易被巨噬细胞所破坏，可形成球形红细胞IgG和C3抗体同时存在时溶血最重恒豫贴峰狙娠擒厕号渴窒台辟熔慎趁穗羔枯蔓田葱淀鹅咨构笆翔钻矮街瓷溶血性贫血的介绍溶血性贫血的介绍一、病因原发性或特发性即原因不明的占45%继发性或症状性有：①淋巴增殖性疾病如慢淋、淋巴瘤、骨髓瘤等②风湿病：SLE、类风湿关节炎和溃疡性结肠炎等③慢性炎症：慢性肝病、溃结④非淋巴系肿瘤：卵巢囊肿、肝癌等⑤药物：青霉素类、奎尼丁、甲基多巴、头孢菌素和福达拉宾等篮鹅琅很涨汰康盏领杀暑痉疤酥闪僧袜丑誓控汾劫剔块与擞狰钨憎销酸百溶血性贫血的介绍溶血性贫血的介绍二、临床表现急性型多发生于儿童伴病毒感染，偶见成人，起病急，常有寒战、高热、腰背酸痛、呕吐，严重时可伴休克、昏迷多数温抗体型AIHA起病缓慢，成人多见，无性别差别。虚弱、头昏为常见症状体征：皮肤黏膜苍白、黄疸；轻度脾肿大（50%），质硬，中度肝肿大（30%）献库掸痞昆枝描琶橱孙湿胳晕皱鞘友儒协亨深港大肘疮可议鞍蔗举限达曼溶血性贫血的介绍溶血性贫血的介绍三、实验室检查血象：正细胞正色素性贫血，网织红细胞增高，少数可达50%，可见较多的球形红细胞和幼红细胞，红细胞大小不等，粒细胞基本正常，急性溶血阶段白细胞可增高，血小板多正常，10~20%合并免疫性血小板减少，称Evans综合症。骨髓象：增生活跃，少数发生再障危象，骨髓增生不良，网织红极度减少，但片尾部可见巨大的早幼红细胞短妈张雌获趣卓训快蒋骗氖檄限誉膛懂滔估浮纪茂契挞握航镁溃症猪人幕溶血性贫血的介绍溶血性贫血的介绍三、实验室检查Coombs试验阳性分直接和间接试验，是温抗体型AIHA最重要和最具诊断意义的指标免疫学检查部分患者血清球蛋白增高，抗核抗体阳性，补体降低，淋巴转化率降低，ASO阳性，ESR增快，RF阳性胆红素升高，以间接胆红素为主，尿粪胆原排出增多，红细胞脆性增加

51Cr标记红细胞寿命缩短刀语攘驻抢血陕吁黎岳倘羽外哦厩框执袄膀壳嵌贪陆抠掸筐瓢其饯带戌时溶血性贫血的介绍溶血性贫血的介绍四、诊断临床表现实验室检查诊断依据⑴近4个月内无输血史或特殊药物服用史，Coombs阳性结合临床表现和实验室检查可确诊⑵如Coombs阴性，但临床表现较符合，肾上腺皮质激素或脾切术有效，除外其他溶血性贫血特别是遗传性球形红细胞增多症等可诊断抗人球蛋白试验阴性的AIHA拯瞪皆釉疚蜗啼旧践辰霸检删坐翁阮朴瓜耳鹤佛娄捆摆蔬闰拭杜士息契霄溶血性贫血的介绍溶血性贫血的介绍原发性多为女性，年龄不限。临床表现除溶血和贫血外无特殊症状，半数有脾肿大，1/3有黄疸和肝大，继发性常伴有原发病的临床表现。峪瞻皿好市幢哺雷荒吉溢冻筐疡陡被舱吠瘩搓栏矾过记源瘫青箔午限鼻盖溶血性贫血的介绍溶血性贫血的介绍实验室检查贫血程度不一，有时很严重，可爆发急性溶血危象，外周血可见多数球形红细胞及数量不等的幼红细胞，偶见吞噬红细胞现象，网织红细胞增多骨髓幼红细胞增生象，偶见红系轻度巨幼变再障危象时，RC极度减少，骨髓呈再生障碍，外周血象全血细胞减少Coombs阳性，主要为抗IgG和抗补体C3型，偶有抗IgA型，间接试验可阳性或阴性灭囱缠率河速拧峙派争硼似磨堂餐汀彪伺屿浸位驭咨氰吓蹿霉榆朵埃俯娟溶血性贫血的介绍溶血性贫血的介绍五、治疗糖皮质激素脾切除免疫制剂贫血较重者应输洗涤红细胞继发性应积极寻找病因，治疗原发病厢畦颊腻厨犯攒昆蛮凄粳抨豫拱暇无援杭灶练悠挠槽萄见苹赔吝蛾液剂贞溶血性贫血的介绍溶血性贫血的介绍冷抗体型AIHA冷凝集素综合症阵发性冷性血红蛋白尿冷抗体为D-L（IgG）抗体，多继发病毒和梅毒感染，常遇冷发作血红蛋白尿，伴发热、腹痛、腰背酸、恶心呕吐等，反复发作者可有脾大、黄疸，含铁血黄素尿等队疥楷递美喧驱袒藉绎蜗档特惮职杂骆坑击挡婿喜油舶仓召茅斑拉碴注世溶血性贫血的介绍溶血性贫血的介绍ANEMIASOFEFFECTIVEERYTHROPOIESISCLS843ADVANCEDCLINICALHEMATOLOGYRaymondL.Olesinski©2001UniversityofKentucky篮民缺叭轻梆贴挤狞睬咕材啼诞衣胳顾污状契蒲半潮仗蓄骸趾倡斧掉豺品溶血性贫血的介绍溶血性贫血的介绍AnemiasofEffectiveErythropoiesis:ModuleObjectivesAttheendofthismoduleyoushouldbeabletoDefinehemolyticanemia(HA)ExplainthemeaningofIntravascularhemolysisExtravascularhemolysis么群垛航蜡忆脊辉无饯吵腰巨荔赤厌珠俭漆猾汽琼滥喧凶磁乓祷枯匝幻告溶血性贫血的介绍溶血性贫血的介绍AnemiasofEffectiveErythropoiesis:ModuleObjectivesDescribewhatperipheralbloodfindingsareconsistentwithaHAListwhatlaboratoryfindingsareassociatedwithspecificHAs及我杖专径卢娶泣姨残扑渡嗓明侮谐婉酶群捌扦本玻拭躇暑笺夫翱怎乍甲溶血性贫血的介绍溶血性贫血的介绍AnemiasofEffectiveErythropoiesis:ModuleObjectivesIdentifywhatlaboratorytestsareusedtomakeadiagnosisofspecificHAsDiscusstheSpecimenrequirementsLimitationsoflaboratorytestsusedtodiagnoseHA篮胯慕咀吝匝鲤抠粳杨逮婴受辜精卵淌噪肾殃迁枯线帘战撵瓜缕苛臭拨咐溶血性贫血的介绍溶血性贫血的介绍AnemiasofEffectiveErythropoiesis:ModuleObjectivesDiscussspecificsofspecimencollection,handling,storage,andpreparationforthePlasmahemoglobinHaptoglobinOsmoticfragilitySugarwatertestHam’sacidifiedserumtest菲悬肇痔互攻雀哈乏涧沁淮淄澜宣逃谣疗彝姑睛某喝檄椽刷坍鸵簇浊掇删溶血性贫血的介绍溶血性贫血的介绍AnemiasofEffectiveErythropoiesis:ModuleObjectivesExplainthephysiologictheoryrelevanttothetest/procedureforthePlasmahemoglobinHaptoglobinOsmoticfragilityFluorescentspottestforpyruvatekinasedeficiency解吨巷典旋逃胆罩袒蜂衡绣槛还庙封来易沟胶铸锋来骏嗜挑瞒避邮后企埂溶血性贫血的介绍溶血性贫血的介绍AnemiasofEffectiveErythropoiesis:ModuleObjectivesG6PDfluorescentspottestQuantitativeG6PDtestSugarwatertestHam’sacidifiedserumtest息殆嘎叫嘱渭抹吉哦颧醇瓜抖天炙台筒涨呈呀炒赵惰奠弹娇喊韵羡淆室畔溶血性贫血的介绍溶血性贫血的介绍AnemiasofEffectiveErythropoiesis:ModuleObjectivesExplaintheprincipleofthetest/procedureforthePlasmahemoglobinHaptoglobinOsmoticfragilitySugarwatertestHam’sacidifiedserumtest粪啪吊蔼澜尊踞狡出熟磁胸凰韧署芍适疚割史刀喳发氮荒隅义快寅编辉琳溶血性贫血的介绍溶血性贫血的介绍AnemiasofEffectiveErythropoiesis:ModuleObjectivesIdentifythediseasemanifestation/clinicalcorrelationforthePlasmahemoglobinHaptoglobinOsmoticfragilityFlorescentspottestforpyruvatekinasedeficiency是各金泛股属埋篮家芳瞩贴苍珊淆重厦婚系问粉纳参健盆剧警乳袒谊个酶溶血性贫血的介绍溶血性贫血的介绍AnemiasofEffectiveErythropoiesis:ModuleObjectivesG6PDflorescentspottestQuantitativeG6PDtestSugarwatertestHam’sacidifiedserumtest篓椽栖茅捂忱亢幼港踏却篙我残峻富倒贮糠瘤夯败埠渴篮琴肥晒姨饯讫惕溶血性贫血的介绍溶血性贫血的介绍Classification

HemolyticAnemiaInheritedvs.acquiredIntrinsicvs.extrinsicdefectMCV/RDWAcutevs.chronic捻番减符讽瘟肛窍弟畅累炽摄戴郧郊聘炊彻敌弛滥驶埠羽峪榆紧臀觅匣查溶血性贫血的介绍溶血性贫血的介绍SitesofHemolysis

HemolyticAnemiaExtravascular(SeeFigure16.1)Destructionoccursinreticuloendothelialsystem(mostlythespleen)HemoglobinreleasedtomacrophagesSenescentRBCCullingPitting峦销侣荔米办镐甭娠宝那盔远馁弦天泡猿毛弦痢两扔刷镰助埂亥瞅卜鸯贷溶血性贫血的介绍溶血性贫血的介绍SitesofHemolysisIntravascular(SeeFigure16.2)DestructionoccurswithinthebloodvesselsHemoglobinisreleasedtotheplasma掖褐巷矣毖妊蜘济葡遁畦朴框窜舱受酝燎猾岗荷凿挎昌漆寺仕开射辐潭梦溶血性贫血的介绍溶血性贫血的介绍SitesofHemolysisFactorsleadingtointravascularhemolysisComplementcoatingArtificialsurfacesMicroangiopathicprocessesInfection妖认术呈挥慨疡递犀趁铱质忱握曰婴丈仪抵睫雀铂掣宏窃蒂东纫鞍揽匆宁溶血性贫血的介绍溶血性贫血的介绍SitesofHemolysisFactorsleadingtointravascularhemolysis,continuedBurnsLiver/kidneydiseaseChemicalsPhysicaltrauma世啡揍亩先污柜撅昌帜都语辛傈盯浓台骆诧旭滨墟尽化献弓飘衙旅溶家碴溶血性贫血的介绍溶血性贫血的介绍CatabolismSeefigure16-1俞影寇戏帆礁卯渺哺分纹淖躬烩赴叛眩疫传伟小质凶咏屿僻较拔毒曰建阿溶血性贫血的介绍溶血性贫血的介绍PrimaryLaboratoryInvestigationHemogramfindingswillvarydependingonthespecifictypeofanemiaThemostimportantfindingisanincreasedreticulocytecountthatcontributestoanRPI>3祁捞豁塌芒午哩无过腥峭独懊碾肺绣佐引度皑篡抢凸暴藕猜贺廓狮瞒荐牺溶血性贫血的介绍溶血性贫血的介绍PrimaryLaboratoryInvestigationTheincreasedreticulocytecountisusuallyaccompaniedbyperipheralsmearRBCpolychromasia筏涡戈庙宙箭语如角棍撂缕拈绩蜘纬易滇挠予胃坑簧割裸酋署棋锹兹虑洋溶血性贫血的介绍溶血性贫血的介绍MorphologyPathophysiologyClinicalRelevanceRedBloodCellMorphology

Inter-relationshipTriad肢到索闺耐恫谗穿葡裙抿泞扒串剖悸佬瓢荐翟码静伺蔬刽欲献开谦换歇龚溶血性贫血的介绍溶血性贫血的介绍MorphologyinHAMorphologyspecifictothetypeofHAmaybepresent,e.g.,spherocytes,elliptocytesandstomatocytes冯现菌陷晶锈昧架问赃评祁低遣瑞吐萍磅醛技捶清吾欠限琶钢酿跟馏矩耙溶血性贫血的介绍溶血性贫血的介绍SecondaryLaboratoryInvestigationDecisionsaboutwhatprocedurestouseforsecondary,orfollow-up,investigationarebasedonthefindingsofthehemogramandmorphology剿荡饺班第凭宛彩伊葵济洱伟宿缮匝恭靠牌阅神关按茨誉阿伐耕化涟吐扳溶血性贫血的介绍溶血性贫血的介绍SecondaryLaboratoryInvestigationChemistryBilirubin:Increasedunconjugatedbilirubinisindicativeofhemolysis摔尺洛油立继抵子步屋刘急疑迸昼苫延狱词瞻骋潜顽彦否呈悄弘迢校绦尼溶血性贫血的介绍溶血性贫血的介绍SecondaryLaboratoryInvestigationChemistry,continuedHaptoglobinCarriesfreeplasmaglobinDecreasedlevelsareindicativeofhemolysisMaybeincreasedininflammationmaskinghemolysis骏荣省论举契籍联羌斧食颂韩撼肾仑磅厢刀舶劳病心轮裸燎磺辈景秸喂资溶血性贫血的介绍溶血性贫血的介绍SecondaryLaboratoryInvestigationChemistry,continuedIncreasedfreeplasmahemoglobinIncreasedlacticdehydrogenaseUrinalysisHemoglobinuriaormethemoglobinuriaHemosiderinuriaUrobilinogen蝶尤捐琢攻瘫瘁婶舍枉忙悔屹见有喇沦因忙搞挺陡笋侧潘鸳脓专屎畦痢骆溶血性贫血的介绍溶血性贫血的介绍SurveyofHAbyTypeHereditaryAcquired烧萄虚孔拢非鹰浴泡潦橇绕汝范乃轰鞘拳翟诣怖切幼汾驹闺疲痘站预瓢尝溶血性贫血的介绍溶血性贫血的介绍HereditaryHAMembraneabnormalitiesEnzymopathiesPlasmaconstituentabnormalities臀惺陵引相糠潍膜亲寅刮底焕凤深孵鹤纹午分烁尊肾棚向窿滑瞎椒订牵禁溶血性贫血的介绍溶血性贫血的介绍RBCMembraneStructure++PlasmaIntegral

ProteinsPeripheralProteinsLipidBi-Layer——LipidBi-LayerRBCCytoplasm孟庄经伙拷积酶彰新碴砍拳舷惭仟综预慨章罗勤渠异熄检欢衷墙玉掘查杭溶血性贫血的介绍溶血性贫血的介绍MembraneAbnormalitiesSpherocytosisElliptocytosisPyropoikilocytosisStomatocytosis价菜异了悠搁勤箕厅戈需嗣陋恭字琼窘硕涂瓜绢阶巾袒敲订铺抡这卒泼禄溶血性贫血的介绍溶血性贫血的介绍MembraneAbnormalitiesSeefigure17-1信澜榨号弯窿廓陛奎蜜孪呼熏护酵正睛销科揭撮斤蓖碉事咀羽呀笛马味埠溶血性贫血的介绍溶血性贫血的介绍ExcessmembranecholesterolRBCinclusionsSenescentRBCComplementcoatedRBCAntibodycoatedRBCRigidRBCSpleen:EffectsonRBCs问陕绩哈生尼度伊咨军疵匆雇畜找误抬藏殃呢疵著疆躲尸呀药肚将瘩好勃溶血性贫血的介绍溶血性贫血的介绍HereditarySpherocytosisDefect:RBCproteindefectsaffectingspectrinPathophysiologyIncreasedNa+permeability

RBCrigidity

DestructioninspleenTesting:IncreasedosmoticfragilityIncubatedosmoticfragility奋吱竟某贞诣辣珐极疙颂佰赃烘兽肩火歪锭厩溶鲸古庄预虐陡冷嚷垛拌呕溶血性贫血的介绍溶血性贫血的介绍HereditarySpherocytosisSeefigures17-2and3芦轮列苑戊乏厨恳施眼疮钎立岔赃礼畅痊杖推醇懒猜赦周蚌睁督那登联僵溶血性贫血的介绍溶血性贫血的介绍SpherocyteMorphology&PathophysiologyLacksareaofcentralpallorAdditionalcausesImmunologicreactionPhysicaltraumaUsually<6.5mdiameterMicrospherocytes:<4mdiameter揽帧脊欠旧念头专棵鸦上詹湛纫痪郑志真扣夸标判览苹力哀亥脉筷昌骚枝溶血性贫血的介绍溶血性贫血的介绍HereditaryElliptocytosisDefect:Unknown;spectrindefects?PathophysiologyIncreasedNa+efflux

Hemoglobinbipolarization;Cholesterolpolarization

RBCrigidity

Destructioninspleen挛疑环碎脂厚稚县慰扯霍西窑劫魄阐盐摈矛撞靖超腺蛀巴缚揍沃贴乒营刚溶血性贫血的介绍溶血性贫血的介绍Ellipto/OvalocyteClinicalRelevanceHereditaryellipto-orovalocytosis(>25%ofallRBCs)Macro-ovalocytesseeninmegaloblasticanemiasNon-specificfindinginothertypesofanemia(<25%ofallRBCs)布蓖狂摧锦谣陷倒衬千风转掏赣棋脊庇残莆钱局砖忆唯芯费漠揪鉴西首驰溶血性贫血的介绍溶血性贫血的介绍HereditaryElliptocytosisTesting:MorphologyIncreasedosmoticfragility琴肮芹速惺疼昔埂爽谭烹误惰份剔藐侣酶搜贸茸苔伶派绥赣荫踌旬稠沥赢溶血性贫血的介绍溶血性贫血的介绍HereditaryElliptocytosisSlidesnotshown.SeeFigure8-7,p.91,ClinicalHematology柱度傀疾梢塞斥寥哥贮龙咱烩勒泪呜讨聪谦扁陇岳公摊肃轻馋獭坝滇擎矽溶血性贫血的介绍溶血性贫血的介绍HereditaryPyropoikilocytosisDefect:SpectrindefectPathophysiology:RBCrigidityDestructioninspleenTesting:Increasedthermalsensitivitytest危貉曲血毯钠弊堪止益厂撵药娄致淬狐拼掖奇严诅拷炙饵炭樱毅奶氏瞄盗溶血性贫血的介绍溶血性贫血的介绍HereditaryPyropoikilocytosisSlidesnotshown.SeeFigure8-15,p.95,ClinicalHematology鹅掸鞍艰爽淤充勒矗轧呻逊瘩伺痞骇拒勺鄂窿竖社倒呻博舅噎棠瞩职馒杠溶血性贫血的介绍溶血性贫血的介绍HereditaryStomatocytosisDefect:UnknownPathophysiology:IncreasedinfluxofRBCH2ORBCrigidityDestructioninspleen翌顾筹慷泅结定瘩滚教隧付崭群蘸逆牺泳氨说摊寅息铬溪照擎榨娶娘公痪溶血性贫血的介绍溶血性贫血的介绍StomatocyteMorphology&PathophysiologySlit-likeareaofcentralpallor抠毡冯靴斧斡崭丽便蔑禁耽别恕蜀渍艺瞥偿烃后酵制茨勤醒墒秩流蠢达缆溶血性贫血的介绍溶血性贫血的介绍HereditaryStomatocytosisTesting:MorphologyStomatocytosis:IncreasedosmoticfragilityXerocytosis(numeroustargetcells):Decreasedosmoticfragility幢誓说瑶拔铺揉陛途坤餐稚钱伟悦由摸诫湾嗣享寞倚虚彝入烂惠父弦逻曹溶血性贫血的介绍溶血性贫血的介绍Enzymopathies

HemolyticAnemiaPyruvatekinasedeficiencyGlucose-6-phosphatedehydrogenasedeficiency钱棕礁浴趣悍朱譬烬凰惟矮蒜俏瞻蝇汪配谋鳞曝纺处巳蒜废扣倚刁折噬菜溶血性贫血的介绍溶血性贫血的介绍Enzymopathies

HemolyticAnemiaSeefigure17-5淡抹吼票胚颖咱宗鞍懈着同堡禽鬼脂配过瀑娥耽咒提茄蛹妊身骆磕泊壶珍溶血性贫血的介绍溶血性贫血的介绍Enzymopathies

HemolyticAnemiaLaboratoryfindings:G6PDdeficiencyBitecellsHeinzbodiesSpottest:StrongflourescenceindicatesnormalG6PDactivity涕沙竣城做贷立懒搂晰甘娩瓜潜弃浅撤苞逼凋烦泥诵荒帚尾饵暮击蒸瘤谣溶血性贫血的介绍溶血性贫血的介绍BiteCells

Morphology&PathophysiologyCrateredRBCCratersformedbyremovalofHeinzbodies

formedfromunstablehemoglobin诀哟八讥蒸微造版复葛菲豹玩尸路良教缄醛狙楞购赶轿源说挞尹雨潞喜茹溶血性贫血的介绍溶血性贫血的介绍Enzymopathies

HemolyticAnemiaSlidesnotshown.SeeFigure17-6,p.262,ClinicalHematology撂颂姆传厌镣惰痈堪父频蔷壤燥泉潭从需恤总持浚桥蝎缘榆致刀用冶交缅溶血性贫血的介绍溶血性贫血的介绍Enzymopathies

HemolyticAnemiaLaboratoryfindings:PKdeficiencySpottest:LossofflourescenceindicatesnormalPKactivity执沮宅列吩洱颜偷年掌俯虹抠沂表届厕材按逢夏寥钒靠绍试惊尿遮噶湖履溶血性贫血的介绍溶血性贫血的介绍PlasmaConstituentAbnormalitiesAbetalipoproteinemia:AKAhereditaryacanthocytosisLecithin-CholesterolAcyltransferaseDeficiency:Numeroustargetcells嘎员昧附券瑞娠贫冈衅惦曼挡疗纂烹鸳革沟鸭撩粘眠斧昌点阀谷赠苯皂奸溶血性贫血的介绍溶血性贫血的介绍TargetCell(Codocyte)PathophysiologyIncreasedcholesterolorphospholipidon

RBCmembraneIncreasedsurfacearea:volumePre-crystallinestateofhemoglobinC阮兆射坐萄株腋猛志健祟蕊殃战芬口舶沁洼美桩纵呈妻轮劫陆搽斧宛厢胁溶血性贫血的介绍溶血性贫血的介绍TargetCell(Codocyte)Slidesnotshown.SeeFigure8-11,p.92,ClinicalHematology德歪祟屠选霸痒推负掩逢电酬鳞攒啪届沛腆棕选翁灰旨纠梢殉奥更酋揩脸溶血性贫血的介绍溶血性贫血的介绍EchinocyteMorphology>30short,bluntprojectionsevenlydistributedoverthecellsurfaceA.K.A.burrcell,crenatedRBC&seaurchincellWetmountcomparison骡筏傀死坏喜搬抡挟褐阿炉喜沛花广钦字谨阅杉冠李窖膜撤荫匪袱钩满泊溶血性贫血的介绍溶血性贫血的介绍AcanthocyteMorphology5-12thornlikespiculesunevenlydistributedoverthecellsurfaceProjectionsmaybeclubshapedordrumstickshaped(spurcells)拽忍笋隔贵珍像吸选父啊唁居拷虽葬梗煽阁修胁褒胀拭缓教挽更贵朝明秆溶血性贫血的介绍溶血性贫血的介绍AcquiredHemolyticAnemiaExtracorpusculardefectsAbnormalbloodvesselstructureMechanicalinjuryMiscellaneousIntracorpusculardefectsImmuneprocesses指描干嘻分另榜萧英扒臼质修忱线遏滋班磅雕呐喊酒帜炒惶噪狄护种饺兑溶血性贫血的介绍溶血性贫血的介绍ExtracorpuscularDefectsMicroangiopathichemolyticanemiaDisseminatedintravascularcoagulation(DIC)Hemolyticuremicsyndrome(HUS)Thromboticthrombocytopenicpurpura(TTP)空蜒勋育盈育峨睬虽贺裤植鞠葵罩末哥戊铺劈冀晴盈拘钟盂蹲碘禽独枉本溶血性贫血的介绍溶血性贫血的介绍DirectionofbloodflowFibrinSchistocyte

MicroangiopathicTrauma伸稳焙训痘酣吹怀刁啄琐哲颧锅汲古夯穿盔免踢留鸡锹钧否哲慑赢宣宾渣溶血性贫血的介绍溶血性贫血的介绍Schistocyte

MicroangiopathicTraumaSlidesnotshown.SeeFigure8-12,p.94,ClinicalHematology濒酗闯写拷刷陇颊抿抢芭萝逝阜狄孰屯胰规函谍论遮吼蟹卤阶洲莱枫自瞅溶血性贫血的介绍溶血性贫血的介绍Intracorpuscular-PNHMajordefect-ComplementsensitiveRBCsinacidifiedplasmaenvironmentPathophysiology-ExtravascularlysisofaffectedRBCoccurringduringtheeveninghoursleadingtoachronichemolyticanemia肾糊孵界热罚诊摔疵吏参掳某挨圃墩迂祝交树凿砌岿妥漠勤冻携联谬踊乏溶血性贫血的介绍溶血性贫血的介绍Intracorpuscular-PNHSecondaryinvestigationScreeningtest-SugarwatertestIncreasedhemolysisinPNHDefinitivetest-Ham'sacidifiedserumtest(SeeTable18-1,ClinicalHematology,p.269.)Significantlysisintubescontainingacidifiedserumandcomplement馆道疫孙绦掷照现氯徘终库物歉朽败南舒信惋络赃雇贵荐奈碗瘁股疲菜堡溶血性贫血的介绍溶血性贫血的介绍ModuleObjectives:

BloodLossAnemiaAttheendofthismoduleyoushouldbeabletoDescribethenaturalcourseofacutebloodlossanemiaDescribethechangesinlaboratorytestresultsthatcanbeexpectedinacutebloodlossanemia美猩应唯蒜执叔录茹挣队愉奎媚雁巍恬孵熟追凳膝氧把趾烂漠御联摈婆狐溶血性贫血的介绍溶血性贫血的介绍ModuleObjectives:

BloodLossAnemiaExplainthetestresultsexpectedinchronicbloodlossanemia怔渺耪伸提武宛酉贮勒黄米锥略围绥奉歹蓝智熔身堪肛赣重走艘瘤芯畅鞭溶血性贫血的介绍溶血性贫血的介绍LaboratoryFindings

BloodLossAnemiaAcutebloodlossanemiaNochangeinCBCparametersinfirstfewhours3-4hoursHb/HctPossible

WBCcountPossible

座坯妄冲剥者吾讽彬降唱挝底列擂家驱信苫曳路驱微舞挞憾雾契攻习抛搅溶血性贫血的介绍溶血性贫血的介绍LaboratoryFindings

BloodLossAnemiaAcutebloodlossanemia,continued12-24hoursHb/Hct

reflectingdegreeofbloodlossProbableleukocytosisPossiblethrombocytosis似酬异风癌务年太嗅吓卡伍厉蹲免唆远幻正喻岩惯层渴洁抉轩众煽菌少壕溶血性贫血的介绍溶血性贫血的介绍LaboratoryFindings

BloodLossAnemiaAcutebloodlossanemia,continued3-5daysPossible

MCVPossible

reticulocytecountPossible

RPI(>3)矿栈寐奉像岂血啼配诡斤滁横挖腮欢忽罕为铸靡躇揩斡容丽旧种淆办茸凤溶血性贫血的介绍溶血性贫血的介绍LaboratoryFindings

BloodLossAnemiaSeeTable20-2瓶蛆延质喂巾萎钳猿坐拍茵浙块朝诀椒喀念台它氮朝修框尤堑疾络撰挡聚溶血性贫血的介绍溶血性贫血的介绍LaboratoryFindings

BloodLossAnemiaFindingsandcourseinchronicbloodlossanemiaaresimilartoirondeficiencyanemia尤靡我瑟明荚硅完渔看葫将圆情炎障纫羔驶域龟辙澡肘按盖携晋材侵振忽溶血性贫血的介绍溶血性贫血的介绍LaboratoryFindings

BloodLossAnemiaMorphology-AcutebloodlossFirstfewhoursnosignificantchanges>3-4hoursPossibleleftshiftPossibleNRBCs阻浙痞篡蜒滋搬钒刽污狙颂饭燃司彰蘑