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How to treat myelodysplastic syndrome with clinical features resembling Behçet syndrome: a case-based systematic review.
Annals of Hematology ( IF 3.5 ) Pub Date : 2020-03-05 , DOI: 10.1007/s00277-020-03951-5
Umut Yilmaz 1 , Muhlis Cem Ar 1 , Sinem Nihal Esatoglu 2 , Isil Bavunoglu 3 , Yusuf Ziya Erzin 4 , Ali Ibrahim Hatemi 4 , Suleyman Yildirim 4 , Gülen Hatemi 2 , Aykut Ferhat Celik 4
Affiliation  

The association between myelodysplastic syndrome (MDS) and Behçet syndrome (BS) is recognized for over 25 years. High frequency of trisomy 8 and intestinal ulcers are striking features of this association. There are no recommendations for how these patients should be treated. A systematic literature review was performed in PubMed using the keyword combination "(((((intestinal) OR gastrointestinal) OR ulcer) OR Behcet*)) AND ((myelodysplastic syndrome) OR MDS)" in March 2019. Our aim was to gain insight regarding clinical responses to individual treatment modalities. A recent case was also presented and included in the analysis. Data from 41 articles reporting on a total of 53 patients carried adequate information to assess treatment responses. Glucocorticoids provided benefit in 23 of 43 patients. Azacitidine, decitabine, thalidomide, and cyclosporine contributed to a clinical improvement in 4/6, 2/3, 3/4, and 5/8 patients respectively. Hematopoietic stem cell transplantation was successful in 9 of 13 patients. With the use of TNF inhibitors, azathioprine, and mesalamine derivatives, clinical improvement was observed in 3/11, 0/4, and 6/18 patients respectively. Patients with MDS and BS-like features who are resistant to glucocorticoids have so far benefited more from treatment approaches directed at MDS, rather than the immunosuppressive agents used for BS.

中文翻译:

如何治疗具有类似Behçet综合征的临床特征的骨髓增生异常综合征:基于病例的系统评价。

骨髓增生异常综合征(MDS)与Behçet综合征(BS)之间的关联已有25年以上的历史了。三体性8和肠溃疡的高频率是这种关联的显着特征。没有关于如何治疗这些患者的建议。2019年3月在PubMed中使用关键字组合“(((((肠道(肠道)或胃肠道)OR溃疡)OR Behcet *))AND((骨髓增生异常综合征)OR MDS)”进行了系统的文献综述。我们的目标是关于对个体治疗方式的临床反应的见解。还介绍了一个最近的案例,并将其包括在分析中。来自41篇报道总共53例患者的数据携带了足够的信息来评估治疗反应。糖皮质激素使43例患者中的23例受益。阿扎胞苷,地西他滨,沙利度胺,环孢素和环孢素分别有助于4 / 6、2 / 3、3 / 4和5/8患者的临床改善。造血干细胞移植在13例患者中有9例成功。通过使用TNF抑制剂,硫唑嘌呤和美沙拉敏衍生物,分别在3 / 11、0 / 4和6/18患者中观察到临床改善。迄今为止,对糖皮质激素有抵抗力的MDS和BS样特征的患者受益于针对MDS的治疗方法,而不是用于BS的免疫抑制剂。和6/18患者。迄今为止,对糖皮质激素有抵抗力的MDS和BS样特征的患者受益于针对MDS的治疗方法,而不是用于BS的免疫抑制剂。和6/18患者。迄今为止,对糖皮质激素有抵抗力的MDS和BS样特征的患者受益于针对MDS的治疗方法,而不是用于BS的免疫抑制剂。
更新日期:2020-03-06
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