PNH F A phosphatidylinositol glycan class A PIGA
- Slides: 49
PNH的病理机制 F 磷脂酰肌醇聚糖A类基因异常 [phosphatidylinositol glycan class A (PIG-A) gene abnormality] F 糖化磷脂酰肌醇连接蛋白 [glycosylphosphatidylinositolanchored proteins(GPI-AP)]缺乏: CD 59: membrane inhibitor of reactive lysis, MIRL CD 55: decay-accelerating factor, DAF
红细胞外的致病因素(1) *免疫溶血性(Immunolytic) 温抗体型自身免疫性溶血性贫血 (AIHA caused by warm-reactive antibodies) 特发性(Idiopathic) 继发性或症状性(Secondary or symptomatic) 病毒和支原体感染(Virus and mycoplasma infections) 淋巴增殖性疾病(Lymphoproliferative diseases, LPD) 其他恶性肿瘤( Other malignant diseases) 免疫缺陷状态(Immune-deficiency states) 自身免疫病(Autoimmune disorders) 药物诱发(Drug-induced)
红细胞外的致病因素(2) *免疫溶血性(Immunolytic) 冷抗体型自身免疫性溶血性贫血 (AIHA caused by cold-reactive antibodies) 冷凝集素病(Cold hemagglutinin disease) 特发性(Idiopathic) 继发性(Secondary) 阵发性冷性血红蛋白尿症 (Paroxysmal cold hemoglobinuria) 血型不合的输血 (Transfusion of incompatible blood) 新生儿溶血病 (Hemolytic disease of the newborn)
红细胞外的致病因素(3) *物理因素(Physical agents) 微血管病性溶血性 (Microangiographic hemolytic) 溶血尿毒症综合征(HUS) 血栓性血小板减少性紫癜(TTP) 弥散性血管内凝血(DIC) 人 瓣膜和其他心脏异常 (Prosthetic valves and other cardiac abnormalities) 热损伤(Caused by thermal injury)
红细胞外的致病因素(4) *药物和化学毒物(Drugs and chemicals) 氧化剂(Oxidant) 非氧化剂(Nonoxidant) 芳香化合物(Aromatic) 氯酸盐(Chlorates) 硝酸盐(Nitrates) 顺铂(Cisplatin) 氧(Oxygen) 氢化砷(Arsine, As. H 3) 锑化氢(Stibine, Sb. H 3) 铅(Lead) 铜(Copper) 水(Water)
四、临床表现(1) *急性溶血性贫血的表现 起病急(begin abruptly) 寒战(shaking chills) 高热(high fever) 全身酸痛(pains over the body ) 苍白(pallor) 黄疸(jaundice) 心动过速(tachycardia) 肾功能衰竭(acute renal failure) 休克(shock)
四、临床表现(2) *慢性溶血性贫血的表现 起病缓慢(occurred slowly) 贫血(anemia) 黄疸(jaundice) 脾肿大(splenomegaly) 胆石症(cholelithiasis) 下肢溃疡(leg ulcers) 骨骼畸形(skeletal abnormalities) 再生障碍危象(Aplastic crisis)
红细胞破坏过多的表现 *红细胞生存时间缩短(Decreased RBC life span) *红细胞形态异常(Morphologic abnormalities of RBC) *血清乳酸脱氢酶活性增高(Increased serum LDH) *血浆游离血红蛋白升高(Hemoglobinemia) *血清结合珠蛋白减少或消失 (Reduced or absence of serum haptoglobin) *血红蛋白尿(Hemoglobinuria) *每周血红蛋白浓度减少 10 g/L以上 (Fall in blood Hb level at a rate > 10 g/L)
血红蛋白代谢产物增多的表现 血清非结合胆红素升高 (Increased serum unconjugated bilirubin level) 血清血结素水平下降 (Reduced serum hemopexin level) 高铁血红素白蛋白血症 (Methemalbuminemia) 尿胆原增多 (Increased rate of urobilinogen excretion) 含铁血黄素尿症 (Hemosiderinuria)
骨髓代偿增生的表现 *网织红细胞升高 (Reticulocytosis, polychromatophilia, stippling) *外周血出现幼红细胞、大红细胞 (Erythroblastosis,Macrocytosis) *骨髓红系过度增生 (Erythroid hyperplasia)
用于病因诊断的特殊检查 *渗透脆性试验(Osmotic fragility test) *自溶血试验(Autohemolysis) *Coombs试验(Antiglobulin test) *Ham试验(complement lysis sensitivity assay) *流式细胞仪检测GPI-AP表达:CD 55、CD 59 *RBC特殊形态异常检查(Spherocyte, Elliptocyte, etal) *红细胞酶的检查(G 6 PD、PK等) *血红蛋白电泳(Hb Electrophoresis) *变性珠蛋白小体生成试验(Heinz-body formation)
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