溶血性贫血识别与评估：从溶血证据到病因分型

先看懂四件事：溶血是什么、怎么分类、怎么评估、怎么治疗Hemolytic Anemia

溶血性贫血的判断是一条链条：先确认红细胞破坏增加（网织红细胞、乳酸脱氢酶、结合珠蛋白、间接胆红素），再用外周血涂片与抗体检查找方向，最后落实到具体病因——遗传性红细胞膜与酶缺陷、自身免疫、微血管病、药物或感染相关，治疗策略也随之不同。

发病机理：红细胞破坏增加的两条路径Pathogenesis

溶血的关键区别在于红细胞「在哪里被破坏」：血管内溶血把血红蛋白直接释放到血浆，血管外溶血则由脾脏等器官把红细胞「清除」掉——这决定了结合珠蛋白下降的程度、涂片表现以及治疗方向。

哪些情况需要警惕溶血

两个容易被忽略的问题

怎么检查：从溶血证据到病因分型Assessment

怎么治疗：激素、靶向药与脾切除Treatment

治疗与管理一览

日常管理：诱因、随访与妊娠Daily Management

十个常见误区Ten Common Misconceptions

参考文献与证据来源References & Evidence

本页共 24 条文献，来自国际共识与指南、随机对照试验、荟萃分析与机制研究，链接指向 PubMed。每条结论后的编号对应下方文献；证据不足或结论不一致之处已在正文标注。

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4. 英国血液学会指南（遗传性球形红细胞增多症的诊断与管理）Bolton-Maggs PH, Langer JC, Iolascon A, Tittensor P, King MJ, General Haematology Task Force of the British Committee for Standards in Haematology. Guidelines for the diagnosis and management of hereditary spherocytosis--2011 update. British journal of haematology. 2012;156(1):37-49. pubmed.ncbi.nlm.nih.gov/22055020
5. 英国血液学会指南（G6PD 缺乏的实验室诊断）Roper D, Layton M, Rees D, Lambert C, Vulliamy T, De la Salle B, D'Souza C, British Society for Haematology. Laboratory diagnosis of G6PD deficiency. A British Society for Haematology Guideline. British journal of haematology. 2020;189(1):24-38. pubmed.ncbi.nlm.nih.gov/31991476
6. 综述（蚕豆病与葡萄糖-6-磷酸脱氢酶缺乏）Luzzatto L, Arese P. Favism and Glucose-6-Phosphate Dehydrogenase Deficiency. The New England journal of medicine. 2018;378(1):60-71. pubmed.ncbi.nlm.nih.gov/29298156
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9. 国际共识建议（成人自身免疫性溶血性贫血的诊断与治疗）Jäger U, Barcellini W, Broome CM, Gertz MA, Hill A, Hill QA, Jilma B, Kuter DJ, Michel M, Montillo M, Röth A, Zeerleder SS, Berentsen S. Diagnosis and treatment of autoimmune hemolytic anemia in adults: Recommendations from the First International Consensus Meeting. Blood reviews. 2020;41():100648. pubmed.ncbi.nlm.nih.gov/31839434
10. 中国指南（成人自身免疫性溶血性贫血诊断与治疗）Red Blood Cell Disease (Anemia) Group, Chinese Society of Hematology, Chinese Medical Association. [Chinese guideline for the diagnosis and treatment of adult autoimmune hemolytic anemia (2023)]. Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi. 2023;44(1):12-18. pubmed.ncbi.nlm.nih.gov/36987718
11. 英国血液学会指南（药物诱导与继发性免疫性溶血性贫血的管理）Hill QA, Stamps R, Massey E, Grainger JD, Provan D, Hill A, British Society for Haematology Guidelines. Guidelines on the management of drug-induced immune and secondary autoimmune, haemolytic anaemia. British journal of haematology. 2017;177(2):208-220. pubmed.ncbi.nlm.nih.gov/28369704
12. 多国观察性研究（232 例冷凝集素病的临床特征）Berentsen S, Barcellini W, D'Sa S, Randen U, Tvedt THA, Fattizzo B, Haukås E, Kell M, Brudevold R, Dahm AEA, Dalgaard J, Frøen H, Hallstensen RF, Jæger PH, Hjorth-Hansen H, Małecka A, Oksman M, Rolke J, Sekhar M, Sørbø JH, Tjønnfjord E, Tsykunova G, Tjønnfjord GE. Cold agglutinin disease revisited: a multinational, observational study of 232 patients. Blood. 2020;136(4):480-488. pubmed.ncbi.nlm.nih.gov/32374875
13. 随机安慰剂对照Ⅲ期试验（苏替利单抗治疗冷凝集素病：CADENZA）Röth A, Berentsen S, Barcellini W, D'Sa S, Jilma B, Michel M, Weitz IC, Yamaguchi M, Nishimura JI, Vos JMI, Storek M, Wong N, Patel P, Jiang X, Vagge DS, Wardęcki M, Shafer F, Lee M, Broome CM. Sutimlimab in patients with cold agglutinin disease: results of the randomized placebo-controlled phase 3 CADENZA trial. Blood. 2022;140(9):980-991. pubmed.ncbi.nlm.nih.gov/35687757
14. 英国血液学会指南（血栓性血小板减少性紫癜与血栓性微血管病的诊断与管理）Scully M, Rayment R, Clark A, Westwood JP, Cranfield T, Gooding R, Bagot CN, Taylor A, Sankar V, Gale D, Dutt T, McIntyre J, Lester W, BSH Committee. A British Society for Haematology Guideline: Diagnosis and management of thrombotic thrombocytopenic purpura and thrombotic microangiopathies. British journal of haematology. 2023;203(4):546-563. pubmed.ncbi.nlm.nih.gov/37586700
15. 随机对照试验（卡普赛珠单抗治疗获得性血栓性血小板减少性紫癜）Scully M, Cataland SR, Peyvandi F, Coppo P, Knöbl P, Kremer Hovinga JA, Metjian A, de la Rubia J, Pavenski K, Callewaert F, Biswas D, De Winter H, Zeldin RK, HERCULES Investigators. Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura. The New England journal of medicine. 2019;380(4):335-346. pubmed.ncbi.nlm.nih.gov/30625070
16. 随机对照试验（pegcetacoplan 与依库珠单抗治疗阵发性睡眠性血红蛋白尿症）Hillmen P, Szer J, Weitz I, Röth A, Höchsmann B, Panse J, Usuki K, Griffin M, Kiladjian JJ, de Castro C, Nishimori H, Tan L, Hamdani M, Deschatelets P, Francois C, Grossi F, Ajayi T, Risitano A, Peffault de Latour R. Pegcetacoplan versus Eculizumab in Paroxysmal Nocturnal Hemoglobinuria. The New England journal of medicine. 2021;384(11):1028-1037. pubmed.ncbi.nlm.nih.gov/33730455
17. 随机Ⅲ期试验（糖皮质激素单药与联合利妥昔单抗治疗自身免疫性溶血性贫血）Birgens H, Frederiksen H, Hasselbalch HC, Rasmussen IH, Nielsen OJ, Kjeldsen L, Larsen H, Mourits-Andersen T, Plesner T, Rønnov-Jessen D, Vestergaard H, Klausen TW, Schöllkopf C. A phase III randomized trial comparing glucocorticoid monotherapy versus glucocorticoid and rituximab in patients with autoimmune haemolytic anaemia. British journal of haematology. 2013;163(3):393-399. pubmed.ncbi.nlm.nih.gov/23981017
18. 随机双盲安慰剂对照试验（利妥昔单抗治疗成人温抗体型自身免疫性溶血性贫血：RAIHA）Michel M, Terriou L, Roudot-Thoraval F, Hamidou M, Ebbo M, Le Guenno G, Galicier L, Audia S, Royer B, Morin AS, Marie Michot J, Jaccard A, Frenzel L, Khellaf M, Godeau B. A randomized and double-blind controlled trial evaluating the safety and efficacy of rituximab for warm auto-immune hemolytic anemia in adults (the RAIHA study). American journal of hematology. 2017;92(1):23-27. pubmed.ncbi.nlm.nih.gov/27696475
19. 欧洲血液学协会建议（遗传性溶血性贫血的脾切除）Iolascon A, Andolfo I, Barcellini W, Corcione F, Garçon L, De Franceschi L, Pignata C, Graziadei G, Pospisilova D, Rees DC, de Montalembert M, Rivella S, Gambale A, Russo R, Ribeiro L, Vives-Corrons J, Martinez PA, Kattamis A, Gulbis B, Cappellini MD, Roberts I, Tamary H, Working Study Group on Red Cells and Iron of the EHA. Recommendations regarding splenectomy in hereditary hemolytic anemias. Haematologica. 2017;102(8):1304-1313. pubmed.ncbi.nlm.nih.gov/28550188
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21. 队列研究（8,149 例脾切除后最长 27 年的长期风险）Kristinsson SY, Gridley G, Hoover RN, Check D, Landgren O. Long-term risks after splenectomy among 8,149 cancer-free American veterans: a cohort study with up to 27 years follow-up. Haematologica. 2014;99(2):392-398. pubmed.ncbi.nlm.nih.gov/24056815
22. 队列研究（血管内溶血与多重治疗预测自身免疫性溶血性贫血患者的血栓）Fattizzo B, Bortolotti M, Giannotta JA, Zaninoni A, Consonni D, Barcellini W. Intravascular hemolysis and multitreatment predict thrombosis in patients with autoimmune hemolytic anemia. Journal of thrombosis and haemostasis : JTH. 2022;20(8):1852-1858. pubmed.ncbi.nlm.nih.gov/35555857
23. 国际多中心研究（妊娠与产褥期自身免疫性溶血性贫血）Fattizzo B, Bortolotti M, Fantini NN, Glenthøj A, Michel M, Napolitano M, Raso S, Chen F, McDonald V, Murakhovskaya I, Vos JMI, Patriarca A, Mingot-Castellano ME, Giordano G, Scarrone M, González-López TJ, Trespidi L, Prati D, Barcellini W. Autoimmune hemolytic anemia during pregnancy and puerperium: an international multicenter experience. Blood. 2023;141(16):2016-2021. pubmed.ncbi.nlm.nih.gov/36706358
24. Cochrane 系统评价（指导红细胞输注的输血阈值）Carson JL, Stanworth SJ, Dennis JA, Trivella M, Roubinian N, Fergusson DA, Triulzi D, Dorée C, Hébert PC. Transfusion thresholds for guiding red blood cell transfusion. The Cochrane database of systematic reviews. 2021;12():CD002042. pubmed.ncbi.nlm.nih.gov/34932836