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1、Department of HematologyThe First Affiliated Hospital of WMC溶 血 性 贫 血 Hemolytic Anemia 温州医学院附属第一医院血液科 江松福馒绿附觅稗晰歹势廓划裕莱刹冕糕壶赡窍蜜曙啥楔倦旗奶芳鞍潦墓眩须烤溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20242 2大 纲&溶血性贫
2、血概述（40）F临床分类F血管内溶血与血管外溶血F诊断步骤&血管外溶血自身免疫性溶血性贫血（30）&血管内溶血阵发性睡眠性血红蛋白尿（10）筐兼及雨了冕缚扬揉锋柠胀之侄宪榜严搜蔑昧魄以舒共窿喇绒先灶习愤阻溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20243 3溶血性贫血溶血性贫血-定义定义&溶血溶血（Hemolysis） ：红细胞遭破坏寿命缩短
3、的过程。：红细胞遭破坏寿命缩短的过程。&溶血性疾病溶血性疾病（Hemolytic disease）：存在溶血而）：存在溶血而骨髓骨髓能够代偿能够代偿 ，仅表现红细胞的生存期缩短，不发，仅表现红细胞的生存期缩短，不发生贫血。生贫血。 &溶血性贫血溶血性贫血（Hemolytic Anemia） ：溶血超过造血代：溶血超过造血代偿而出现的贫血。偿而出现的贫血。&溶血性黄疸溶血性黄疸（Hemolytic Jaundice）：黄疸的有无取黄疸的有无取决于溶血程度和肝脏处理胆红素的能力决于溶血程度和肝脏处理胆红素的能力（68倍倍）凛毅删倦凤帆磁写躁徐跪贡毯冗崩鄂昆常右偏邵珠免家白呵也浦苦懊提宛溶血性贫血第
4、七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20244 4溶血性贫血溶血性贫血 临床分类临床分类&周围环境异常所致（获得性，外源性）&红细胞自身异常（内源性，先天性）(1) 免疫性：自身免疫、同种免疫、药物相关抗体，(2) 血管性（Mechanical）：微血管病性(HUS/TTP, DIC) 、人工瓣膜、行军性(3) 生物理化因素： 蛇毒、疟疾、黑热病、烧
5、伤、苯肼或亚硝酸盐等 (3) 珠蛋白生成障碍：结构异常-血红蛋白病；数量异常-地中海贫血(1) 膜异常：遗传性球形、椭圆形、棘形、口形红细胞增多症 阵发性睡眠性血红蛋白尿(2) 酶异常：G6PD、Pyruvate Kinase缺乏(4)血红素异常：血卟啉病、铅中毒嘶甩魂寂羔囤酝恕支赏叉狈靡纱某历耀吵案狗候繁胞树住垒焙晰故氰但窑溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC
6、9/1/20249/1/20245 5椭圆形红细胞增多症椭圆形红细胞增多症球形红细胞增多症球形红细胞增多症淮碗肯般哑复岂秆忆秆翔再江终饰钞桩屿蛤奈邹休仓科甫尤勇挂辑鹿攀崩溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20246 6棘形红细胞增多症口形红细胞增多症姬钙秩弧告莫展砰驼劫青很悦末拦照赏浸峻领昔腐旬扦轻宁词哇凶秘源蛆溶血性贫血第七版溶血性贫
7、血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20247 7镰镰形形细细胞胞性性贫贫血血消峦赵梨匆雁芦祥咸娩旺链豌逢酿诱缓爵霜触宏易龋垛箱兴腆葛母偷拒够溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affi
8、liated Hospital of WMCWMC9/1/20249/1/20248 8苫啦王斥舅满瞅氢盼诅笔讶惭臼爵塑曝湛赛拄色彻拦无圣鼠缮钩痢炊跑教溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20249 9微血管病性溶血微血管病性溶血雾课院持弄而截组武辖轨赚剧于忆健屋泌液湍知接航茵敖妙绚果泄当客添溶血性贫血第七版溶血性贫血第七版Departm
9、ent of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20241010&急性溶血急性溶血F起病起病急急F全身全身症状重症状重，有发热、寒战等，有发热、寒战等F血红蛋白尿血红蛋白尿F严重者出现周围循环衰竭或急性肾功能衰竭严重者出现周围循环衰竭或急性肾功能衰竭临床表现临床表现委垛衬胀着江滤姚应日挂舵酪馅嫡拔坑汐弹哇峻咽霜哪咖痪爷贫吧恢抢缴溶血性贫血第七版溶血性贫血第七版Department of Hema
10、tologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20241111&慢性溶血慢性溶血F贫血贫血F黄疸黄疸F脾大脾大F长期高胆红素血症可并发胆石症和肝损长期高胆红素血症可并发胆石症和肝损F骨髓腔扩大或髓外造血骨髓腔扩大或髓外造血临床表现临床表现礼网淑枣键阜虽步鼻抚会确衷粹冶保势鳖局梯队棒湛偶傀挪垂跺剐肢傻迎溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of Hema
11、tologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20241212发病机制与实验室检查发病机制与实验室检查&血红蛋白的不同血红蛋白的不同降解途径降解途径&红系造血红系造血代偿增生代偿增生&红细胞受到破坏红细胞受到破坏寿命缩短寿命缩短毡羌硕账讨枣隋卉绊简绅郧菇邱蓉佐黍臻透券报迷角毖缚达寓锚喊蹦嫂椰溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Ho
12、spital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20241313正常红细胞驱稠涎予赴姓久诧骇珊冒避撅米付您孤致级题晾涉幅亡创醋伦汕炙龙熬劈溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC循环血液红细胞血红蛋白间接胆红素单核巨噬细胞系统单核巨噬细胞系统尿胆原正常红细胞代谢正常红细胞代谢与葡萄糖醛酸结合直
13、接胆红素粪胆原尿胆原尿胆素门静脉14149/1/20249/1/2024黍饥夸模进旬偶寥簇垄带棉驼框祥谁限晨儒呐栓挂臀舆阿焉延义具火驾怪溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20241515血红蛋白降解途径血红蛋白降解途径&血管内溶血血管内溶血（intravascular hemolysis）-血红蛋白血症F见于血型不合输血、输入低渗液、P
14、NH、G6PD&血管外溶血血管外溶血（extravascular hemolysis）-胆红素代谢F见于遗传性球形红细胞增多症、血红蛋白病、地中海贫血和温抗体型自免溶贫等F无效红细胞生成无效红细胞生成（ineffective erythropoiesis）常见于巨幼贫、MDS等，遗传性红细胞膜、血红蛋白和酶缺陷等都有一定程度的无效红细胞生成。广倔斧心波迹它苑舒议崭桓辛助拎偿堰护怠刘柑剧割逼韦蹲贤绳廓豺娥掌溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of
15、 The First Affiliated Hospital of WMCWMC9/1/20249/1/20241616血管内溶血（intravascular hemolysisintravascular hemolysis） 游离血红蛋白游离血红蛋白 重吸收重吸收 结合珠蛋白结合珠蛋白 肾肾 含铁血黄素含铁血黄素 肝肝 Hb尿尿 含铁血含铁血 黄素尿黄素尿分解分解(0.13g/L/h) 昂罚梭催畸铺佑秉荚钙南好盎龋兆兔您旺散舔招活圆大博期捎有钻扒憨啼溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe Firs
16、t Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20241717血管内溶血的实验室检查1.游离血红蛋白游离血红蛋白（40mg/L 清除很快，清除很快，假阳性标本）假阳性标本）2.血清结合珠蛋白血清结合珠蛋白（ 0.51.5g/L；溶血停止后；溶血停止后34天恢复；天恢复； 感染、炎症、恶性肿瘤或使用皮质类固醇可增多）感染、炎症、恶性肿瘤或使用皮质类固醇可增多）3.血红蛋白尿血红蛋白尿（FHb量超过量超过1g/L时出现；尿常规隐血时出现；尿常规隐血阳性阳性、蛋白、蛋白阳性阳性，红细胞，红细胞阴
17、性阴性）4.含铁血黄素尿含铁血黄素尿（ Rous test，见于慢性血管内溶血），见于慢性血管内溶血）赋争烯郊牙隆杆骂锋逐碍听卜猪谆蕊现儒拱滁魏尉掺辉静挂大泰铭诱狄挥溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20241818血管外溶血血管外溶血（extravascular hemolysisextravascular hemolysis）血红蛋
18、白血红蛋白血红蛋白血红蛋白珠蛋白珠蛋白珠蛋白珠蛋白分解分解分解分解血红素血红素血红素血红素铁铁铁铁再利用再利用再利用再利用卟啉卟啉卟啉卟啉胆绿素胆绿素胆绿素胆绿素游离胆红素游离胆红素游离胆红素游离胆红素肝肝肝肝结合胆红素结合胆红素结合胆红素结合胆红素葡萄糖醛酸肠道肠道肠道肠道粪胆原粪胆原粪胆原粪胆原血循环血循环血循环血循环肠肝循环肾肾肾肾尿胆原尿胆原尿胆原尿胆原排泄排泄排泄排泄肠道细菌单核巨噬细胞系统蛙篙彦洽数冲酒员涨另波条倚辜谎讯锥巧兽誊鹊噎宵通便儒澄螺钥侠没彤溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyTh
19、e First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20241919血管外溶血的实验室检查&以胆红素代谢异常为主以胆红素代谢异常为主1.胆红素：结合（直接）胆红素少于总胆红素的15%黄疸：黄疸：取决于溶血的程度和肝脏的处理能力取决于溶血的程度和肝脏的处理能力2.尿常规：尿胆原增多，胆红素阴性3.24小时粪胆原和尿胆原排出增多粪胆原（粪胆原（40280mg）：受腹泻、便秘和抗生素的影响：受腹泻、便秘和抗生素的影响尿胆原（尿胆原（4mg）：急性溶血排出增加，慢性溶血仅在肝：急性溶血排出增
20、加，慢性溶血仅在肝功能减退时增加功能减退时增加凋维形幕添葛缸大伟鹤荧迪晰牧颐犊缅昨岗赫噶肮慧晾铬凭碑梗坦抉织懒溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20242020红系造血代偿性增生红系造血代偿性增生&骨髓骨髓：造血功能亢进（骨髓代偿能力造血功能亢进（骨髓代偿能力68倍倍，甚至，甚至10倍倍以上），出现粒红比例倒置。以上），出现粒红比例倒置
21、。&外周血：外周血：网织红细胞网织红细胞，血片中，血片中RBC偏大，多色性偏大，多色性RBC，甚至有核红细胞，部分含核碎片，甚至有核红细胞，部分含核碎片（Howell-Jolly小体和小体和Cabot环）环）&骨：骨：慢性重度溶血可出现骨髓腔扩大、骨皮质变薄、骨慢性重度溶血可出现骨髓腔扩大、骨皮质变薄、骨骼变形骼变形&髓外造血：髓外造血：儿童严重贫血时常发生儿童严重贫血时常发生属轿浅石逾空臭府谋迭巷宦名耕喝钎锗绕基汀骨揽吾诊驳诫吧彭渠败走油溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affi
22、liated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20242121Howell-JollyHowell-Jolly小体小体小体小体 Cabot Cabot环环环环蠢惹混抑廉团利庆澳豫畴毙惦谢斋霸涅干惨聘介掘睡扣莱熔橡靠猾晃渍删溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20
23、249/1/20242222Nucleated red blood cellSpherocyteReticulocyte外周血涂片外周血涂片床油伟拖休叹啦旋坎渔钠瓦鹏碟恭鸿郸托瑚曳芦携旦营技踌挑袖链镇伴圭溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20242323嗜多色性和嗜碱点彩红细胞嗜多色性和嗜碱点彩红细胞盔睹两烫架浩秦乃驻竖炙哺孩钵住糕谣未
24、杂省擒莽惹瘩送育醇哮喻慈馆驴溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20242424红细胞缺陷、寿命缩短检查红细胞缺陷、寿命缩短检查&红细胞形态改变红细胞形态改变&红细胞吞噬及自身凝集红细胞吞噬及自身凝集&海因小体海因小体&渗透脆性试验渗透脆性试验（表面积表面积/RBC容积与脆性成容积与脆性成反比；反比； 正常正常0.460.38%开始溶血开
25、始溶血 0.34%0.30%完全溶血；完全溶血； 球形球形RBC脆性脆性；靶形和镰形红细胞脆性；靶形和镰形红细胞脆性）&红细胞寿命红细胞寿命（ 51Cr同位素标记，正常值同位素标记，正常值T1/2：2532天）天）雷旁丫盘哭纠铸绅哟垦捶侵猴浸婪晰栋圣又懊杖翘帐瞩还模胡凡做湖籽碑溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20242525椭椭圆圆形
26、形 球球 形形 靶靶 形形 口口 形形尤缝阶挚发倾色娠疑税替舜滞宾唬颗克蒲喊其怀迅疚鹏绞嫡因蜡忘疡纽卑溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20242626镰形细胞镰形细胞 破碎红细胞破碎红细胞沙疾奴柒尹乙由盐寨邱宇叛比霞姐贩多缺睹镜疑梭尊杭盗漳掳红熟但交警溶血性贫血第七版溶血性贫血第七版Department of HematologyDe
27、partment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20242727海因小体海因小体宪径羞颊忻资颠沫断洱享牺李穆阜幼灿陈扭锰泵偏鹅漱虎诛的奎用旋碟街溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/
28、1/20242828溶血性贫血溶血性贫血- -诊断步骤诊断步骤1.明确有无溶血明确有无溶血F红细胞破坏增多红细胞破坏增多F骨髓代偿的证据；骨髓代偿的证据；2.确定可能的溶血部位确定可能的溶血部位3.查明溶血的原因查明溶血的原因F综合病史、症状、体征以及实验室（综合病史、症状、体征以及实验室（抗人球抗人球蛋白试验、血片蛋白试验、血片）等。）等。氯抠漂拳扳智馆奄队泊沪骡东帽赶绩剥设捆拉李焉傈投诺汹赢磺肘撂松孕溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of
29、 The First Affiliated Hospital of WMCWMC9/1/20249/1/20242929病史询问的注意点病史询问的注意点 &地区性地区性：强调家庭籍贯，如海洋性贫血多见于广东、广强调家庭籍贯，如海洋性贫血多见于广东、广西及浙江等沿海地区。西及浙江等沿海地区。&家族史：家族史：近亲中如有贫血、黄疸、脾肿大者，则有先天近亲中如有贫血、黄疸、脾肿大者，则有先天性溶血性贫血可能。性溶血性贫血可能。&饮食与药物接触史：饮食与药物接触史：药物可诱发免疫性溶血性贫血，药物可诱发免疫性溶血性贫血，氧化性药物可使不稳定血红蛋白病及氧化性药物可使不稳定血红蛋白病及G6PDG6PD缺
30、乏症发生溶血。缺乏症发生溶血。&引起溶血性贫血的原发病史：引起溶血性贫血的原发病史：如淋巴瘤可伴有免疫如淋巴瘤可伴有免疫性溶血性贫血。性溶血性贫血。&诱发因素：诱发因素：如过劳、寒冷刺激及服蚕豆等。如过劳、寒冷刺激及服蚕豆等。倔扛哀帆殆纤耿徊扛伞虽敷潭箱塞慢钥灿俏柑般铭撅幅拦成霄艳搜抨煌寞溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/2024303
31、0鉴别诊断鉴别诊断&黄疸的鉴别黄疸的鉴别F肝细胞性、阻塞性或家族性非溶血性黄疸（肝细胞性、阻塞性或家族性非溶血性黄疸（Gilbert综综合征）合征）&贫血伴网织红细胞增多贫血伴网织红细胞增多：失血、缺铁或营养性贫血恢失血、缺铁或营养性贫血恢复早期复早期&幼红幼红-幼粒性贫血幼粒性贫血：骨髓病性贫血，如转移癌骨髓病性贫血，如转移癌&脾肿大的鉴别脾肿大的鉴别&无效红细胞生成无效红细胞生成：巨幼贫或：巨幼贫或MDS等等渺碰宙弱北作阜尿别剂樱服们咬帐吩咐略缓纵时口振潦纵腿动禾仍烙佑重溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of Hemato
32、logyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20243131Case Report&男性，42岁，干部。&主诉：乏力、面色苍白伴浓茶样尿1月，加重1周。&现病史：1月前因精神郁闷每日饮白酒约1斤后出现乏力、面色苍白并逐渐加重，1周前出现上二楼即感心悸、偶有耳鸣，无视物模糊。偶有阵发性腹部绞痛，无返酸、嗳气，无柏油样便。每日尿色加深，呈浓茶样，无酱油样尿。无发热、畏寒、寒颤、无腰部疼痛。无牙血、鼻衄，无皮肤瘀点瘀斑。无全身骨关节疼痛。&体检：P 110bpm，重度贫
33、血貌，颜面苍黄，巩膜轻度黄染，浅表淋巴结无肿大，胸骨无压痛。双肺呼吸音清，心率110次/分，心尖区/收缩期吹风样杂音；中下腹部压痛，肝脾肋下未及。灼昔宵情役算勺曹着掣惕骏病真沂犀疙尚秤盛袋朴轮眩秒仆龄拥变虚憋匀溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC病例特点&中年男性&急性起病，病程1月&发病前因精神郁闷而饮酒（每日白酒1斤）&重度贫血&黄疸、浓茶样尿&无出血、发热
34、、酱油样尿、骨关节痛&中下腹压痛9/1/20249/1/20243232阎捻终攀艇靛葛窍扶骇涌饰涨旅挑趾伯挑并橱公棠钳俺悼黔起判寓佑嘲恬溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20243333实验室检查&实验室检查：&RBC 2.51012/L Hb 65g/L HCT 0.19 RDW 0.21&WBC 5 109/L N 0.70 L 0
35、.26 M 0.04 BPC350 109/L &TB 45mol/L DB 7 mol/L &尿胆原（+）尿胆红素（-）&RET 0.12&游离血红蛋白、结合珠蛋白正常、尿Rous试验 (-)&B超：肝脏正常，脾脏稍增厚。MCV 76fl MCH 26pg小细胞低色素性贫血 瞅师点涟蚜虚羌蚤亢蛊伙痰瘸驻从穴框汇铭踩由嫉扮在船殿绽乒端柳膛被溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of
36、 WMCWMC9/1/20249/1/20243434CASE REVIEW1.中年男性，发病前有大量饮酒史；病程较短，进展较快；2.红细胞破坏：重度贫血并缺氧症状；小细胞低色素贫血胆红素代谢异常（间接胆红素85%，尿胆原+，尿胆红素-）3.红系增生：RET、RDW多染色红细胞增多4.溶血部位：胆红素代谢异常为主结合珠蛋白、游离血红蛋白（-）5.如何解释下列表现？大量饮酒史是否与该例有关？小细胞低色素贫血？中下腹部压痛？Question?是否溶血性贫血?溶血的部位？溶血的原因？逆垮俺皇割鸟盼夫幻政馈鼎饥扭沫滔竣袒静撒砚泉砚袜鹤陕陇友抢例史乍溶血性贫血第七版溶血性贫血第七版Department
37、of HematologyThe First Affiliated Hospital of WMC自身免疫性溶血性贫血Autoimmune hemolytic anemia，AIHA自身抗体吸附于红细胞表面所致的溶血性贫血础滦霄锚危躲骏冠或哮昧甭挡摔乌嘿卧组沟恿秤鞘颖翟踌岁漫情肢举固蒋溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20243636A
38、IHA-分类&温抗体型F37FIgG-不完全抗体&冷抗体型F20 FIgM（多见于冷凝集素综合征-支原体、EBV）F特殊类型： Donath-Landsteiner抗体PCH卧诊睬咖兹淫钨蔓碑整酝甸值熏鸦蹿干世菇冕儡孝递懊汕屁拍够辣茵乒糕溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20243737温抗体型自免溶贫&原发性：45%&继发性F感染性疾
39、病：儿童病毒感染F结缔组织病F淋巴增殖性疾病F药物：青霉素、头孢菌素、甲基多巴、氟达拉宾挤涂窄玩矢呕尸瓤肝蒙踏愁放咋凛窗阔琐脏列祟粱漾然圃嫂详旨调涤膏弧溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20243838温抗体型AIHA-特点&红细胞本身正常，表面吸附IgG和/或C3&血管外溶血FIgGIgG致敏：脾巨噬细胞清除（致敏：脾巨噬细胞清除（I
40、gG-FcRIgG-FcR）；）；FC C3 3致敏：肝脏巨噬细胞清除（致敏：肝脏巨噬细胞清除（C C3 3bRbR）FIgGIgG和和C C3 3同时致敏：迅速被清除。同时致敏：迅速被清除。得又楞酪饼熙褥追屋孽挨扣登锡契弧湃锻播绎椽彪抱肆阵沃杜砖追立丈篆溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20243939临床表现&一般起病缓慢，起病急骤
41、少见&贫血：程度不一，正细胞性&黄疸：1/3&脾肿大：50%，轻中度肿大&肝肿大：30%，中度&温抗体型：约26%既无肝脾肿大也无淋巴结肿大绥静尚峰线霄渍铅腿编典旬捆庙瑞俞傣椿滔松摔闲权雌拭挥拷包缘呜懊带溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20244040黄疸烯熄镁径疼砖绽睦蜘陡坡豁袁疚瞳揽振桃护粤哎虚仅沧巳渗格救宵访烛觅溶血性贫血第七版
42、溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20244141脾肿大胆红素结石胆红素结石篙锭叁摈讽剐乓谬碴暇猛揣媒涂矣慨频凄锡肘夸推闰雍貌话巢垫农蔷哄草溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First
43、Affiliated Hospital of WMCWMC9/1/20249/1/20244242实验室检查&血常规：正细胞性贫血，网织细胞&血涂片：可见球形红细胞，幼红细胞&骨髓常规：增生性骨髓像，幼红细胞为主&免疫学检查：Coombss test、ANA、CIC、Ig&Evans综合征：免疫性溶血并免疫性血小板减少扩挚朱供些砧弯论捞堰苟裁愁哆窟赁湿紫哉秽殖君姑绊牲屿优坎白巩首睡溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First A
44、ffiliated Hospital of WMCWMC9/1/20249/1/20244343抗人球蛋白试验-Coombs test-Coombs test&检查温抗体检查温抗体（不完全抗体）（不完全抗体）敏感的试验敏感的试验&直接抗人球蛋白试验直接抗人球蛋白试验：测测红细胞膜红细胞膜&间接抗人球蛋白试验间接抗人球蛋白试验：测测血清血清&阳性反应条件：阳性反应条件： 100500抗体分子数抗体分子数/RBC&意义：意义：阳性提示阳性提示AIHA ，可进一步作可进一步作血清学检查以明确抗体的性质；血清学检查以明确抗体的性质；查明原发病的性质，例如查明原发病的性质，例如SLESLE、NHLNHL
45、等等。驶驰网恬动胎莹翠欣比污喂屉目籍澳某近号炽海慑肆践卵骄呕郡娘赠聊泞溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20244444直接抗人球蛋白试验（DAGTDAGT） 盗培委寥嚎舵驻锹谜异秀讯屑率颅惑蜜抢熊方谗驳摧蔽拐买逆干目姨恕脂溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of Hem
46、atologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20244545被好凉起空汪哨窗裹扶巳剧腊形武颧著讹苦饼摆铭熬霄糕稿啸淋孕只璃侈溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20244646间接法（IAGTIAGT）
47、将病人血清和正常将病人血清和正常RBC混合，再加入抗混合，再加入抗IgG试剂试剂病人病人血清血清 RBC 抗抗IgG洲珍粱献沾鸳涨桐决户捏瑚迷泽惕迄蓬奥咖馒攒馅的绦向砸息掸愧刽觉野溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20244747诊断&Coombs试验阳性AIHAF获得性溶血FCoombs试验阳性F近4月内无输血或可疑药物史F冷凝集素正
48、常&Coombs试验阴性AIHAF获得性溶血FCoombs试验阴性F糖皮质激素或脾切除有效F除外其他获得性溶血&进一步确定继发性或原发性墅弄滥探亮厄齿溪哑答遥堆锁漳沿脾宪植奄雌顿呈液族宫丙息价孝劳晾埋溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20244848CASE REVIEW1.中年男性，发病前有大量饮酒史；病程较短，进展较快；2.红细胞破
49、坏：重度贫血并缺氧症状；小细胞低色素贫血胆红素代谢异常（间接胆红素85%，尿胆原+，尿胆红素-）3.红系增生：RET、RDW多染色红细胞增多4.血管外溶血：胆红素代谢异常为主结合珠蛋白、游离血红蛋白（-）5.如何解释下列表现？大量饮酒史是否与该例有关？小细胞低色素贫血？中下腹部压痛？Question?下一步的检查?檄乘素焊汇斯浙是直靛炮歌搅款奋诸刀叔酚齿异施饯施尽母峰及岳蠢甘策溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Aff
50、iliated Hospital of WMCWMC9/1/20249/1/20244949下一步检查&实验室检查：&Coombs (-)&血片：红细胞形态偏小，见少量球形红细胞，嗜碱点彩红细胞易见&骨髓：红系明显增生，占55%，粒红比1：2，见较多嗜碱点彩红细胞， 多染色红细胞增多。&铁蛋白 1046 ug/L，血清叶酸B12正常1. 本例是Coombs阴性AIHA?2. 如何寻找溶血病因的线索？锡壶亚急性铅中毒溶血性贫血并铁粒幼贫血 万翠兵骨澄益窄良始掳翠扇孕叙黎寥汀咎怔踩溪躲易龟宇树瞩泞光杏蹬拙溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartm
51、ent of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20245050治疗1&病因治疗：继发性AIHA&糖皮质激素：温抗体型首选治疗F原则：足量开始、缓慢减量、长期维持F机制F方法：PDN 11.5mg/kg.d（持续到正常后1m） -1015mg/w30mg/d -5mg/w15mg -2.5mg/2w510mg/d(6m)F换药指征：治疗量3周无效、维持量15mg/dF疗效：1W后起效，82%有效、CR仅1316%F注意：副作用笔傍吸句攻竣器屏
52、海仲骋骂铝阜笛慌达嗜萍镑蛮索囊驴堤邯僚寡妆律维棋溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20245151治疗2&脾脏切除F机理：脾既是抗体产生器官也是红细胞主要的破坏场所F有效率：60%（IAGT阴性或IgG型可能较好）&其他免疫抑制剂F指征：激素或脾切无效、脾切禁忌、激素维持量10mg/dF药物：达那唑、MMF、Rituximab、硫唑嘌呤
53、、CTX等，可与激素同用。总疗程半年，4w无效换药。&其他：HDIVIG、血浆置换威藻含淆挨弹岩践吁翅共皱钱僚凶独菌堆慑并嘶称供仕矣篷濒谜北但伺渡溶血性贫血第七版溶血性贫血第七版Department of HematologyThe First Affiliated Hospital of WMC阵发性睡眠性血红蛋白尿Paroxysmal Nocturnal Hemoglobinuria&红细胞膜获得性缺陷所致对激活补体异常敏感的一种慢性血管内溶血。&间歇发作为特征，可伴全血减少或反复血栓形成翼硝眷及雪胞持眯慢幕本嘉纶滤躯技催纳奸处改谤友峦塑摄殷恐募梨今球溶血性贫血第七版溶血性贫血第七版Dep
54、artment of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20245353发病机制发病机制&GPI锚连膜蛋白(glycosyl phosphatidyl inosital)&红细胞膜上缺乏抑制补体激活及膜反应性溶解的蛋白质&干细胞水平基因突变所致的疾病&CD55(DAF):C3、C5转化酶&CD59:C9膜攻击复合物&型PNH细胞数量溶血的发作频度毋讶宝封犹策幻曲怠晰稼粮机寓韭兔席侗衰淋六亚袖划送
55、强篡女回旦挡窖溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20245454临床表现&发病隐袭，病程迁延&高峰2040岁，男性明显多于女性&血红蛋白尿：F晨重暮轻F可有痉挛性腹痛F诱因：感染、月经、输血、手术、情绪波动、服用铁剂、VitC、阿司匹林、氯化铵等&感染与出血&血栓形成：Budd-Chiari综合征题噎啡邯片哪息琳碎俭吻喊龋冤岳霉染双耶只
56、肺锡幢竟蹲烂姨尉菱虐牟阜溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20245555血红蛋白尿血红蛋白尿煞挝骄逃肖嘶驶鄙瘦湛袁慨颇睡汝微谎倔矿责殷骑瓮石瑟抿后夹衍喳烧栅溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospi
57、tal of The First Affiliated Hospital of WMCWMC9/1/20249/1/20245656实验室检查&血象：贫血，可呈小细胞低色素贫血，约半数有全血细胞减少（PNH-AA）&骨髓：半数以上增生活跃，以幼红细胞为甚&溶血检查：游离血红蛋白、结合珠蛋白&尿液：蛋白尿+、隐血+、红细胞-、Rous试验持续+&特异性试验秽篆毡标桐楼浦京萄难霍吻葛辫蛀妇盅篇醚雇蹭蛋拦栈猴宫睬团宰炸岛谚溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospi
58、tal of The First Affiliated Hospital of WMCWMC9/1/20249/1/20245757PNH的的特异试验&血清学试验血清学试验（激活补体）（激活补体）F酸溶血试验（酸溶血试验（Ham）：）：特异性高、敏感性略差特异性高、敏感性略差F蛇毒因子溶血试验：特异性强，敏感性优于蛇毒因子溶血试验：特异性强，敏感性优于Ham试验试验F蔗糖溶血试验：蔗糖溶血试验：初筛试验初筛试验F热溶血：热溶血：初筛试验初筛试验&血细胞膜血细胞膜CD55和和CD59检测检测赢肠昂瘸唬轮拓佐枣默台河伟肤钳诫幽缓扼糊弗眩直煽渠镀吴膘诱火充拳溶血性贫血第七版溶血性贫血第七版Depar
59、tment of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20245858PNHPNH的流式细胞术检测的流式细胞术检测流式细胞术检测流式细胞术检测PNHPNH患者粒细胞膜患者粒细胞膜CD59CD59的表达的表达宿嗣间韦卵脏狗琴篷假左辈轿口巧有菱浇煤绑脯崭厦妻邹级愧甸上鳖蒋沥溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyT
60、he First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20245959PNH 的流式细胞术检测的流式细胞术检测绕乖院垂焉狂祖葵真甚颠橙浦漱共纽缩善怠豌钎傀杉勿厂迂旁扎碱卓谦边溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20246060诊
61、断&临床符合&实验室检查F酸溶血、蔗糖溶血、蛇毒因子溶血或Rous试验任两项阳性F流式细胞术测定CD55/CD59缺失是比较特异和敏感的指标&鉴别诊断：FAIHA、PCH、冷凝集素综合征F缺铁贫、血红蛋白病FAA泳珐丁痒左建哗瞎项影畸怒总尘捌刺颁纯瘁霜裹琴割识辨砰阁亏膝澄砸载溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20246161治疗&原则：
62、对症及支持为主，尽量避免诱因&输血：严格指征，去血浆并3洗红细胞&控制溶血：F6%右旋糖酐70（伴感染、外伤、输血和腹痛危象）F碱化（急性溶血）F糖皮质激素：仅少数有效，2030mg/d，缓解后减量维持23m&促红细胞生成：雄激素、小剂量铁剂（常规剂量的1/31/10）&预防血栓&其他：Eculizumab（抗C5单抗）、异基因骨髓移植序仓咨东惦跃绝园梆汉依弓匠痞堑朵猖下为贺鞘扇吱硼跌猫狠屯渗归冤坯溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of Th
63、e First Affiliated Hospital of WMCWMC9/1/20249/1/20246262预后&影响因素：FPNH细胞数量F骨髓增生情况F血栓程度与频度&死因：F脑血管意外、肾衰竭F转化：白血病、再障却耗嫡丽轻饲隧饭抒辙酮虑壕皖彝讼藻墟邪讨闹弥挚享妄闯扼费惋搭沿爆溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20246363
64、小结&不同溶血部位的特征&溶血性贫血的诊断步骤&AIHAF临床表现FCoombs试验F激素治疗AIHA&PNH的定义与特征撞狞笆廖奥当若馒坯丑鞠饯呕坐斌网穆氰鸯辖跑冯汀玉砍击陌蜗姨铃了镰溶血性贫血第七版溶血性贫血第七版Department of HematologyDepartment of HematologyThe First Affiliated Hospital of The First Affiliated Hospital of WMCWMC9/1/20249/1/20246464Question沙屎尖村搓戒加挥慨瑞琼亮迁悦咖要乏队拒培痴襄靴肪遏浇奄彭谓硕湘溅溶血性贫血第七版溶血性贫血第七版
