Interpretation of Chinese expert consensus on the diagnosis and treatment of Evans syndrome(2024)
-
摘要: Evans综合征(Evans syndrome,ES)是一种罕见的自身免疫性疾病,其特点是临床病情严重,复发率高,常见感染和血栓性并发症,死亡率高。ES的治疗具有高度异质性,目前尚无前瞻性随机对照试验且缺乏大型回顾性研究证据,仅有少数病例报告。中华医学会血液学分会红细胞疾病(贫血)学组参考国内外最新ES相关文献制定了《Evans综合征诊断和治疗中国专家共识(2024年版)》。为更好地指导临床实践,文章拟对该共识的诊断和治疗部分进行解读。Abstract: Evans syndrome(ES) is a rare autoimmune disease characterized by a severe clinical course, high recurrence rate, frequent complications such as thrombosis and infection, and high mortality. The management of ES is highly heterogeneous. The current literature on ES is limited to a scarcity of prospective randomized controlled trials or large retrospective studies, with only a few case reports. The Red Blood Cell Diseases(Anemia) Group under the Chinese Society of Hematology within the Chinese Medical Association has developed the Chinese expert consensus on the diagnosis and treatment of Evans syndrome(2024), based on the latest literature pertaining to ES. To provide better guidance for clinical practice, this article aims to interpret the consensus regarding diagnosis and treatment.
-
Key words:
- Evans syndrome /
- diagnosis /
- management
-
[1] Audia S, Grienay N, Mounier M, et al. Evans' Syndrome: From Diagnosis to Treatment[J]. J Clin Med, 2020, 9(12): 3851. doi: 10.3390/jcm9123851
[2] Hansen DL, Möller S, Andersen K, et al. Evans syndrome in adults-incidence, prevalence, and survival in a nationwide cohort[J]. Am J Hematol, 2019, 94(10): 1081-1090. doi: 10.1002/ajh.25574
[3] Michel M, Chanet V, Dechartres A, et al. The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases[J]. Blood, 2009, 114(15): 3167-3172. doi: 10.1182/blood-2009-04-215368
[4] Fattizzo B, Michel M, Giannotta JA, et al. Evans syndrome in adults: an observational multicenter study[J]. Blood Adv, 2021, 5(24): 5468-5478. doi: 10.1182/bloodadvances.2021005610
[5] 中华医学会血液学分会红细胞疾病(贫血)学组. Evans综合征诊断和治疗中国专家共识(2024年版)[J]. 中华血液学杂志, 2024, 45(9): 633-639.
[6] Neunert C, Terrell DR, Arnold DM, et al. American Society of Hematology 2019 guidelines for immune thrombocytopenia[J]. Blood Adv, 2019, 3(23): 3829-3866. [published correction appears in Blood Adv, 2020, 4(2): 252]. doi: 10.1182/bloodadvances.2019000966
[7] Jäger U, Barcellini W, Broome CM, 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
[8] Fioredda F, Skokowa J, Tamary H, et al. The European Guidelines on Diagnosis and Management of Neutropenia in Adults and Children: A Consensus Between the European Hematology Association and the EuNet-INNOCHRON COST Action[J]. Hemasphere, 2023, 7(4): e872. doi: 10.1097/HS9.0000000000000872
[9] Fattizzo B, Barcellini W. Autoimmune Cytopenias in Chronic Lymphocytic Leukemia: Focus on Molecular Aspects[J]. Front Oncol, 2020, 9: 1435. doi: 10.3389/fonc.2019.01435
[10] Carli G, Visco C, Falisi E, et al. Evans syndrome secondary to chronic lymphocytic leukaemia: presentation, treatment, and outcome[J]. Ann Hematol, 2016, 95(6): 863-870. doi: 10.1007/s00277-016-2642-x
[11] Zhang L, Wu X, Wang L, et al. Clinical Features of Systemic Lupus Erythematosus Patients Complicated With Evans Syndrome: A Case-Control, Single Center Study[J]. Medicine(Baltimore), 2016, 95(15): e3279.
[12] Costallat GL, Appenzeller S, Costallat LT. Evans syndrome and systemic lupus erythematosus: clinical presentation and outcome[J]. Joint Bone Spine, 2012, 79(4): 362-364. doi: 10.1016/j.jbspin.2011.07.004
[13] Li M, Nguyen CB, Yeung Z, et al. Evans syndrome in a patient with COVID-19[J]. Br J Haematol, 2020, 190(2): e59-e61.
[14] Beydoun SB, Persaud Y, Lafferty J, et al. Bortezomib treatment of steroid-refractory Evans syndrome in children[J]. Pediatr Blood Cancer, 2020, 67(12): e28725. doi: 10.1002/pbc.28725
[15] Crickx E, Audia S, Robbins A, et al. Daratumumab, an original approach for treating multi-refractory autoimmune cytopenia[J]. Haematologica, 2021, 106(12): 3198-3201. doi: 10.3324/haematol.2021.279232
[16] Li M, Liu L, Ding B, et al. Refractory/relapse thrombocytopenia in a patient with Evans' syndrome successfully treated with zanubrutinib[J]. Br J Haematol, 2022, 199(5): e37-e42.
[17] Liu H, Ding K, Zhang W, et al. A pilot study of orelabrutinib treatment in three cases of refractory/relapsed autoimmune haemolytic anaemia/Evans syndrome[J]. Br J Haematol, 2024, 204(3): 1082-1085. doi: 10.1111/bjh.19146
[18] Gudbrandsdottir S, Birgens HS, Frederiksen H, et al. Rituximab and dexamethasone vs dexamethasone monotherapy in newly diagnosed patients with primary immune thrombocytopenia[J]. Blood, 2013, 121(11): 1976-1981. doi: 10.1182/blood-2012-09-455691
[19] Michel M, Terriou L, Roudot-Thoraval F, et al. A randomized and double-blind controlled trial evaluating the safety and efficacy of rituximab for warm autoimmune hemolytic anemia in adults(the RAIHA study)[J]. Am J Hematol, 2017, 92(1): 23-27. doi: 10.1002/ajh.24570
[20] Bader-Meunier B, Aladjidi N, Bellmann F, et al. Rituximab therapy for childhood Evans syndrome[J]. Haematologica, 2007, 92(12): 1691-1694. doi: 10.3324/haematol.11540
[21] 董恂玮, 张立鹏, 赵玉平, 等. 84例成人Evans综合征临床资料分析[J]. 中华血液学杂志, 2010, 31(7): 475-477.
[22] Bride KL, Vincent T, Smith-Whitley K, et al. Sirolimus is effective in relapsed/refractory autoimmune cytopenias: results of a prospective multi-institutional trial[J]. Blood, 2016, 127(1): 17-28. doi: 10.1182/blood-2015-07-657981
[23] Sulpizio ED, Raghunathan V, Shatzel JJ, et al. Long-term remission rates after splenectomy in adults with Evans syndrome compared to immune thrombocytopenia: a single-center retrospective study[J]. Eur J Haematol, 2020, 104(1): 55-58. doi: 10.1111/ejh.13336
[24] Yusuf HR, Hooper WC, Grosse SD, et al. Risk of venous thromboembolism occurrence among adults with selected autoimmune diseases: a study among a U.S. cohort of commercial insurance enrollees[J]. Thromb Res, 2015, 135(1): 50-57. doi: 10.1016/j.thromres.2014.10.012
[25] Lecouffe-Desprets M, Néel A, Graveleau J, et al. Venous thromboembolism related to warm autoimmune hemolytic anemia: a case-control study[J]. Autoimmun Rev, 2015, 14(11): 1023-1028. doi: 10.1016/j.autrev.2015.07.001
[26] Serris A, Amoura Z, Canoui-Poitrine F, et al. Efficacy and safety of rituximab for systemic lupus erythematosus-associated immune cytopenias: a multicenter retrospective cohort study of 71 adults[J]. Am J Hematol, 2018, 93(3): 424-429. doi: 10.1002/ajh.24999
[27] Gobert D, Bussel JB, Cunningham-Rundles C, et al. Efficacy and safety of rituximab in common variable immunodeficiency-associated immune cytopenias: a retrospective multicentre study on 33 patients[J]. Br J Haematol, 2011, 155(4): 498-508. doi: 10.1111/j.1365-2141.2011.08880.x
[28] Rossignol J, Michallet AS, Oberic L, et al. Rituximab-cyclophosphamide-dexamethasone combination in the management of autoimmune cytopenias associated with chronic lymphocytic leukemia[J]. Leukemia, 2011, 25(3): 473-478. doi: 10.1038/leu.2010.278
计量
- 文章访问数: 505
- 施引文献: 0