肝脏疾病合并溶血性贫血的研究进展

王谦; 孔明

doi:10.12449/JCH260905

肝脏疾病合并溶血性贫血的研究进展

DOI: 10.12449/JCH260905

王谦,
孔明^, ,

首都医科大学附属北京佑安医院肝病中心四科，北京 100069

基金项目:

国家科技重大专项 (2025ZD01906404)

利益冲突声明：本文不存在任何利益冲突。

作者贡献声明：王谦负责文献收集、论文框架设计和初稿撰写；孔明负责写作思路拟定、重要学术内容核校、全文修订和最终定稿。

详细信息

通信作者:
孔明， kbstef@163.com （ORCID： 0000-0003-0890-2829）

计量
- 文章访问数: 12
- HTML全文浏览量: 8
- PDF下载量: 0
- 被引次数: 0
出版历程
- 收稿日期: 2026-07-07
- 录用日期: 2026-09-16
- 出版日期: 2026-09-25

Research advances in hemolytic anemia in liver diseases

Qian Wang,
Ming Kong^{,
,}

Fourth Department of Liver Disease Center，Beijing YouAn Hospital，Capital Medical University，Beijing 100069，China

Research funding:

National Science and Technology Major Project (2025ZD01906404)

More Information

Corresponding author: Kong Ming， kbstef@163.com （ORCID： 0000-0003-0890-2829）

摘要

摘要: 肝脏疾病与溶血性贫血存在双向联系。肝脏病变可通过红细胞膜脂质异常、铜毒性作用、氧化应激、机械损伤、免疫异常等机制，诱发或促进红细胞破坏；部分原发性溶血性疾病可因黄疸、胆色素结石、脾大或腹腔静脉血栓形成，首先表现为肝胆系统异常。根据红细胞主要破坏部位，溶血可分为血管内溶血和血管外溶血，但二者并非相互排斥，且可随疾病阶段和治疗情况发生变化。肝豆状核变性急性肝衰竭相关溶血、阵发性睡眠性血红蛋白尿及部分经颈静脉肝内门体分流术术后溶血以血管内破坏为主要特征；棘状细胞贫血、遗传性红细胞膜病和温抗体型自身免疫性溶血性贫血以血管外破坏为主；Zieve综合征、葡萄糖-6-磷酸脱氢酶缺乏症、部分自身免疫性溶血及肝移植术后过客淋巴细胞综合征可兼有两种溶血途径。本文以溶血的发生部位为核心框架，以免疫机制和非免疫机制为补充，综述肝病相关溶血及可表现为肝胆异常的原发性溶血性疾病，并提出分层诊断和病因导向的治疗路径。
- 肝疾病 /
- 贫血，溶血性 /
- 溶血
Abstract: There is a bidirectional relationship between liver diseases and hemolytic anemia. Liver diseases may cause or aggravate erythrocyte destruction through abnormalities in erythrocyte membrane lipids， copper toxicity， oxidative stress， mechanical injury， and immune dysregulation； conversely， some primary hemolytic diseases may initially present with abnormalities in the hepatobiliary system due to jaundice， bile pigment stones， splenomegaly， or thrombosis of the splanchnic veins. Hemolysis is classified into intravascular hemolysis and extravascular hemolysis according to the predominant site of erythrocyte destruction； however， the two processes may coexist and may change with disease stage or treatment. Hemolysis associated with Wilson’s disease and acute liver failure， paroxysmal nocturnal hemoglobinuria， and some cases of hemolysis after transjugular intrahepatic portosystemic shunt are characterized by intravascular destruction； spur cell anemia， hereditary erythrocyte membrane disorders， and warm-antibody autoimmune hemolytic anemia are mainly characterized by extravascular destruction； Zieve syndrome， glucose-6-phosphate dehydrogenase deficiency， some cases of autoimmune hemolytic anemia， and passenger lymphocyte syndrome after liver transplantation may involve both pathways of hemolysis. With the site of hemolysis as the primary framework and the immune versus non-immune mechanisms as a supplementation， this article reviews hemolysis associated with liver diseases and primary hemolytic disorders that may present with hepatobiliary abnormalities and proposes a therapeutic approach based on stratified diagnosis and etiology.
- Liver Diseases /
- Anemia， Hemolytic /
- Hemolysis

HTML全文

参考文献(27)

[1]	Berentsen S, Barcellini W. Autoimmune hemolytic anemias[J]. N Engl J Med, 2021, 385( 15): 1407- 1419. DOI: 10.1056/NEJMra2033982.
[2]	Jandl J H. Hemolytic anemia[J]. N Engl J Med, 1963, 268( 9): 482- 486. DOI: 10.1056/nejm196302282680909.
[3]	Scheiner B, Semmler G, Maurer F, et al. Prevalence of and risk factors for anaemia in patients with advanced chronic liver disease[J]. Liver Int, 2020, 40( 1): 194- 204. DOI: 10.1111/liv.14229.
[4]	Tariq T, Karabon P, Irfan F B, et al. National trends and outcomes of nonautoimmune hemolytic anemia in alcoholic liver disease: Analysis of the nationwide inpatient sample[J]. J Clin Gastroenterol, 2021, 55( 3): 258- 262. DOI: 10.1097/MCG.0000000000001383.
[5]	Roy A, Rodge G, Goenka M K. Spur cell anaemia in cirrhosis: A narrative review[J]. J Clin Exp Hepatol, 2023, 13( 3): 500- 508. DOI: 10.1016/j.jceh.2022.10.005.
[6]	Bevilacqua M, de Marco L, Stupia R, et al. Spur cells in liver cirrhosis are predictive of acute-on-chronic liver failure and liver-related mortality regardless of severe anaemia[J]. Intern Emerg Med, 2023, 18( 5): 1397- 1404. DOI: 10.1007/s11739-023-03303-x.
[7]	European Association for Study of Liver. EASL clinical practice guidelines: Wilson’s disease[J]. J Hepatol, 2012, 56( 3): 671- 685. DOI: 10.1016/j.jhep.2011.11.007.
[8]	Kuei A, Mikolajczyk A E, Niemeyer M M. Hemolytic anemia following stent-graft transjugular intrahepatic portosystemic shunt creation[J]. Semin Intervent Radiol, 2019, 36( 2): 72- 75. DOI: 10.1055/s-0039-1688418.
[9]	European Association for the Study of the Liver. EASL clinical practice guidelines on TIPS[J]. J Hepatol, 2025, 83( 1): 177- 210. DOI: 10.1016/j.jhep.2025.01.029.
[10]	Thompson A J, Fellay J, Patel K, et al. Variants in the ITPA gene protect against ribavirin-induced hemolytic anemia and decrease the need for ribavirin dose reduction[J]. Gastroenterology, 2010, 139( 4): 1181- 1189. DOI: 10.1053/j.gastro.2010.06.016.
[11]	Luzzatto L, Ally M, Notaro R. Glucose-6-phosphate dehydrogenase deficiency[J]. Blood, 2020, 136( 11): 1225- 1240. DOI: 10.1182/blood.2019000944.
[12]	Red Blood Cell Disease(Anemia) Group, Chinese Society of Hematology, Chinese Medical Association. Guidelines for the diagnosis and management of paroxysmal nocturnal hemoglobinuria(2024)[J]. Chin J Hematol, 2024, 45( 8): 727- 737. DOI: 10.3760/cma.j.cn121090-20240624-00232. 中华医学会血液学分会红细胞疾病(贫血)学组. 阵发性睡眠性血红蛋白尿症诊断与治疗中国指南(2024年版)[J]. 中华血液学杂志, 2024, 45( 8): 727- 737. DOI: 10.3760/cma.j.cn121090-20240624-00232.
[13]	Plessier A, Esposito-Farèse M, Baiges A, et al. Paroxysmal nocturnal hemoglobinuria and vascular liver disease: Eculizumab therapy decreases mortality and thrombotic complications[J]. Am J Hematol, 2022, 97( 4): 431- 439. DOI: 10.1002/ajh.26474.
[14]	European Association for the Study of the Liver. EASL Clinical Practice Guidelines on vascular diseases of the liver[J]. J Hepatol, 2026, 84( 2): 399- 456. DOI: 10.1016/j.jhep.2025.08.001.
[15]	Szer J. Extravascular hemolysis in PNH[J]. Blood Adv, 2025, 9( 19): 4977- 4978. DOI: 10.1182/bloodadvances.2025016873.
[16]	Cooper R A, Diloy Puray M, Lando P, et al. An analysis of lipoproteins, bile acids, and red cell membranes associated with target cells and spur cells in patients with liver disease[J]. J Clin Invest, 1972, 51( 12): 3182- 3192. DOI: 10.1172/JCI107145.
[17]	Bolton-Maggs P H B, Langer J C, Iolascon A, et al. Guidelines for the diagnosis and management of hereditary spherocytosis-2011 update[J]. Br J Haematol, 2012, 156( 1): 37- 49. DOI: 10.1111/j.1365-2141.2011.08921.x.
[18]	Andolfo I, Iolascon A, Russo R. The evolving landscape of hereditary stomatocytosis[J]. Blood, 2025, 145( 26): 3089- 3100. DOI: 10.1182/blood.2024024294.
[19]	Jäger U, Barcellini W, Broome C M, et al. Diagnosis and treatment of autoimmune hemolytic anemia in adults: Recommendations from the First International Consensus Meeting[J]. Blood Rev, 2020, 41: 100648. DOI: 10.1016/j.blre.2019.100648.
[20]	European Association for the Study of the Liver. EASL Clinical Practice Guidelines on the management of autoimmune hepatitis[J]. J Hepatol, 2025, 83( 2): 453- 501. DOI: 10.1016/j.jhep.2025.03.017.
[21]	Abdulrasak M, Someili A M, Mohrag M. Cytopenias in autoimmune liver diseases-a review[J]. J Clin Med, 2025, 14( 5): 1732. DOI: 10.3390/jcm14051732.
[22]	Suzuki T, Okamoto T, Kawai F, et al. Hemolytic anemia after acute hepatitis B virus infection: A case report and systematic review[J]. Intern Med, 2022, 61( 4): 481- 488. DOI: 10.2169/internalmedicine.7690-21.
[23]	Nastasio S, Matarazzo L, Sciveres M, et al. Giant cell hepatitis associated with autoimmune hemolytic anemia: An update[J]. Transl Gastroenterol Hepatol, 2021, 6: 25. DOI: 10.21037/tgh.2020.03.10.
[24]	Maggiore G, Sciveres M. Giant cell hepatitis associated with autoimmune hemolytic anemia: More evidence for B-cell depletion therapy for a rare immune mediated disease of infancy[J]. Clin Res Hepatol Gastroenterol, 48( 8): 102435.
[25]	Zieve L. Jaundice, hyperlipemia and hemolytic anemia: A heretofore unrecognized syndrome associated with alcoholic fatty liver and cirrhosis[J]. Ann Intern Med, 1958, 48( 3): 471- 496. DOI: 10.7326/0003-4819-48-3-471.
[26]	Pan Y F, Zhao A P, Jiang X J, et al. Passenger lymphocyte syndrome- Epidemiology, pathogenesis, diagnosis, treatment and future directions: A review[J]. Biomol Biomed, 2025, 26( 2): 215- 226. DOI: 10.17305/bb.2025.12548.
[27]	Audet M, Panaro F, Piardi T, et al. Passenger lymphocyte syndrome and liver transplantation[J]. Clin Dev Immunol, 2008, 2008: 715769. DOI: 10.1155/2008/715769.