血液型白塞综合征临床诊治进展

摘要/Abstract

摘要：

白塞综合征是一种可累及多系统的血管炎症性疾病，累及血液系统时称为血液型白塞综合征。该类型临床罕见，医务人员认知相对不足，易造成漏诊或误诊，患者预后通常较差。本文旨在综述血液型白塞综合征的临床表型特点及治疗策略，以期为临床实践提供参考。

中图分类号:

R593.2

申艳, 管剑龙. 血液型白塞综合征临床诊治进展[J]. 内科理论与实践, 2025, 20(04): 272-275.

SHEN Yan, GUAN Jianlong. Progress in clinical diagnosis and treatment of blood type Behcet syndrome[J]. Journal of Internal Medicine Concepts & Practice, 2025, 20(04): 272-275.

参考文献34

[1]	Müftüoglu AU. Symposium on the hematological and immunological aspects of Behçet’s disease[J].Haematologica,1980,65(3):374-380. pmid:6778790
[2]	Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised international chapel hill consensus conference nomenclature of vasculitides[J].Arthritis Rheum, 2013,65(1):1-11.
[3]	Nehashi Y, Torii Y, Yaguchi M, et al. Incomplete-form of Behçet disease in a case of monopathic myelodysplastic syndrome presenting as a thrombocytopenia[J].Rinsho Ketsueki,1988,29(7):1097-1102. pmid:3054202
[4]	Zou J, Luo JF, Shen Y, et al. Cluster analysis of phenotypes of patients with Behçet’s syndrome: a large cohort study from a referral center in China[J].Arthritis Res Ther,2021,23(1):45.
[5]	Soejima Y, Kirino Y, Takeno M, et al. Changes in the proportion of clinical clusters contribute to the phenotypic evolution of Behçet’s disease in Japan[J].Arthritis Res Ther,2021,23(1):49. doi:10.1186/s13075-020-02406-6pmid:33522943
[6]	She CH, Hu D, Zou J, et al. Clinical phenotypes of adult-onset Behçet’s syndrome: a comprehensive cross-sectional study in China[J].Clin Rheumatol,2024,43(7):2261-2271.
[7]	Shen Y, Ma HF, Luo D, et al. High incidence of gastrointestinal ulceration and cytogenetic aberration of trisomy 8 as typical features of Behçet’s disease associated with myelodysplastic syndrome: a series of 16 consecutive Chinese patients from the Shanghai Behçet’s disease database and comparison with the literature[J].Biomed Res Int,2018,2018:8535091.
[8]	马海芬, 申艳, 罗丹, 等. 白塞病合并8号染色体三体22例分析及文献复习[J].复旦学报(医学版),2021,48(4):457-462.
	Ma HF, Shen Y, Luo D, et al. Analysis of 22 cases of Behcet’s disease associated with trisomy 8 and literature review[J].Fudan Univ J Med Sci,2021,48(4):457-462.
[9]	Zou J, Luo D, Shen Y, et al. Characteristics and phenotype heterogeneity in late-onset Behçet’s syndrome: a cohort from a referral center in China[J].Clin Rheumatol,2021,40(6):2319-2326.
[10]	Park JB, Han SJ, Lee SB, et al. Optimal treatment approaches to intestinal Behçet’s disease complicated by Myelodysplastic syndrome: the KASID and KSBD multicenter study[J].Yonsei Med J,2024,65(5):265-275.
[11]	赵婉雯.19例儿童白塞病样8号染色体三体临床特征分析[D]. 长春: 吉林大学,2024.
	Zhao WW.Clinical features of childhood Behçet’s disease like trisomy 8:a summary of 19 cases[D]. Changchun: Jilin University,2024.
[12]	Ding Y, Hu W, Li L, et al. Clinical features and independent predictors of Behçet’s disease associated with myelodysplastic syndrome[J].Clin Exp Rheumatol,2023,41(9):1823-1830.
[13]	Haase D. Cytogenetic features in myelodysplastic syndromes[J].Ann Hematol,2008,87(7):515-526. doi:10.1007/s00277-008-0483-ypmid:18414863
[14]	Thachil JV, Salim R, Field A, et al. Myelodysplastic syndrome with trisomy 8 associated with Behçet syndrome: an immunologic link to a karyotypic abnormality[J].Pediatr Blood Cancer,2008,50(3):649-651. pmid:17066465
[15]	Kimura S, Kuroda J, Akaogi T, et al. Trisomy 8 involved in myelodysplastic syndromes as a risk factor for intestinal ulcers and thrombosis-Behçet’s syndrome[J].Leuk Lymphoma,2001,42(1-2):115-121.
[16]	Hisamatsu T, Naganuma M, Matsuoka K, et al. Diagnosis and management of intestinal Behçet’s disease[J].Clin J Gastroenterol,2014,7(3):205-212. doi:10.1007/s12328-014-0488-0pmid:24883128
[17]	Chen G, Zeng W, Miyazato A, et al. Distinctive gene expression profiles of CD34 cells from patients with myelodysplastic syndrome characterized by specific chromosomal abnormalities[J].Blood,2004,104(13):4210-4218. doi:10.1182/blood-2004-01-0103pmid:15315976
[18]	Wesner N, Drevon L, Guedon A, et al. Gastrointestinal Behcet’s-like disease with myelodysplastic neoplasms with trisomy 8: a French case series and literature review[J].Leuk Lymphoma,2019,60(7):1782-1788.
[19]	Yamazaki M, Yajima T, Tanabe M, et al. Mucosal T cells expressing high levels of IL-7 receptor are potential targets for treatment of chronic colitis[J].J Immunol,2003,171(3):1556-1563. pmid:12874249
[20]	Lee S, Choi WH, Kim JS, et al. Trisomy 8 associated Behçet’s like disease[J].J Rheum Dis,2021,28(2):107-109.
[21]	Wesner N, Fenaux P, Jachiet V, et al. Behçet’s-like syndrome and other dysimmunitary manifestations related to myelodysplastic syndromes with trisomy 8[J].Rev Med Interne,2021,42(3):170-176. doi:10.1016/j.revmed.2020.08.016pmid:33139078
[22]	Lv Q, Li Y, Wei Q, et al. Autoinflammatory syndromes mimicking Behçet’s disease with gastrointestinal involvement: a retrospective analysis[J].Clin Exp Rheumatol,2024,42(10):2076-2085.
[23]	Zhang X, Zhao Y, Pan Y, et al. Trisomy 8 presentation by inflammatory manifestations and its response to thalidomide: two case reports and narrative review[J].Front Pediatr,2024,12:1431511.
[24]	Fu Y, Wu W, Chen Z, et al. Trisomy 8 associated clonal cytopenia featured with acquired auto-inflammation and its response to JAK inhibitors[J].Front Med (Lausanne),2022,9:895965.
[25]	Okamoto T, Okada M, Mori A, et al. Correlation between immunological abnormalities and prognosis in myelodysplastic syndrome patients[J].Int J Hematol,1997,66(3):345-351. pmid:9401280
[26]	Liu Z, Yang C, Bai X, et al. Clinical features and prognosis of patients with gastrointestinal Behçet’s disease-like syndrome and myelodysplastic syndrome with and without trisomy 8[J].Semin Arthritis Rheum,2022,55:152039.
[27]	Kobayashi K, Ueno F, Bito S, et al. Development of consensus statements for the diagnosis and management of intestinal Behçet’s disease using a modified Delphi approach[J].J Gastroenterol,2007,42(9):737-745. pmid:17876543
[28]	Pan FY, Fan HZ, Zhuang SH, et al. Severe inflammatory disorder in trisomy 8 without myelodysplastic syndrome and response to methylprednisolone: a case report[J].World J Clin Cases,2023,11(26):6206-6212.
[29]	Wei Q, Zhang X, Peng Y, et al. Successful treatment by thalidomide therapy of intestinal Behçet’s disease associated with trisomy 8 myelodysplastic syndrome[J].Rheumatology (Oxford),2021,60(6):e200-e202.
[30]	Vallet H, Riviere S, Sanna A, et al. Efficacy of anti-TNF alpha in severe and/or refractory Behçet’s disease: multicenter study of 124 patients[J].J Autoimmun,2015,62:67-74.
[31]	Bao HF, Hou CC, Ye B, et al. Predictors of infliximab refractory intestinal Behçet’s syndrome: a retrospective cohort study from the Shanghai Behçet’s syndrome database[J].Mod Rheumatol,2023,33(1):207-216.
[32]	Yilmaz U, Ar MC, Esatoglu SN, et al. How to treat myelodysplastic syndrome with clinical features resembling Behçet syndrome: a case-based systematic review[J].Ann Hematol,2020,99(6):1193-1203. doi:10.1007/s00277-020-03951-5pmid:32140893
[33]	Liu C, Fu C, Guo X, et al. Allogeneic hematopoietic stem cell transplantation for juvenile myelomonocytic leukemia with intestinal Behçet’s disease: a case report[J].Int J Rheum Dis,2024,27(1):e14882.
[34]	Soysal T, Salihoğlu A, Esatoğlu SN, et al. Bone marrow transplantation for Behçet’s disease: a case report and systematic review of the literature[J].Rheumatology (Oxford),2014,53(6):1136-1141.

血液型白塞综合征临床诊治进展

Progress in clinical diagnosis and treatment of blood type Behcet syndrome

RichHTML

PDF (PC)

可视化

摘要/Abstract

引用本文

使用本文

参考文献34

相关文章11

编辑推荐

Metrics

本文评价

[1]	徐丽辰, 李贵森.局灶节段性肾小球硬化发病机制及治疗进展[J]. 内科理论与实践, 2023, 18(05): 363-367.
[2]	王凡, 叶俊娜, 杨程德.回纹型风湿症的诊断与治疗进展[J]. 内科理论与实践, 2022, 17(03): 258-261.
[3]	邹峻, 管剑龙.白塞综合征临床表型分类和研究进展[J]. 内科理论与实践, 2022, 17(03): 195-197.
[4]	李雅, 陈瑜, 李军民,.老年急性髓系白血病的治疗进展[J]. 内科理论与实践, 2019, 14(06): 391-394.
[5]	梁媛媛, 王璇, 汤建平.干燥综合征合并肺间质病变的临床诊断治疗进展[J]. 诊断学理论与实践, 2018, 17(05): 606-610.
[6]	赵任, 蒋奕玫,.直肠癌外科手术治疗进展[J]. 外科理论与实践, 2016, 21(06): 468-471.
[7]	彭友多, 尹路,.克罗恩病外科治疗进展[J]. 外科理论与实践, 2011, 16(03): 316-318.
[8]	马俊, 汤睿, 顾岩,.腹股沟复发疝的研究现状及治疗进展[J]. 外科理论与实践, 2010, 15(06): 688-691.
[9]	.互刊信息[J]. 内科理论与实践, 2008, 3(04): 219-.
[10]	王广义,杜晓宏.腹股沟疝无张力修补的治疗进展[J]. 外科理论与实践, 2002, 7(06): 481-482+485.
[11]	张家新,沈镇宙,邵志敏.乳腺导管原位癌目前治疗进展[J]. 外科理论与实践, 2000, 5(01): 57-58.