模拟白塞病的骨髓增生异常综合征2例报道并文献回顾  

Myelodysplastic syndrome mimicking Behcet’s disease:two case reports and literat-ure review

作　　者：邵勤[1] 张华勇[2] 陈智勇[2] SHAO Qin;ZHANG Hua-Yong;CHEN Zhi-Yong(Department of Rheumatology,Chongqing Hospital of Traditional Chinese Medicine,Chongqing 400021,China)

机构地区：[1]重庆市中医院风湿科,重庆400021 [2]南京大学医学院附属鼓楼医院风湿免疫科,南京210000

出　　处：《中国免疫学杂志》2019年第17期2136-2140,共5页Chinese Journal of Immunology

摘　　要：目的:探讨白塞病(BD)合并骨髓增生异常综合征(MDS)的临床特征,减少临床误诊。方法:回顾性分析2017年南京鼓楼医院收治的模拟BD的MDS病例2例,并检索中英文文献库中BD合并MDS的病例,分析其临床特点。结果:BD伴发MDS共60例,男性25例,女性35例。年龄15~77岁,平均(45.1±15.3)岁,其中40~69岁患者共38例,占63.3%。61.7%的患者BD先于MDS诊断。消化道损伤、发热、血细胞减少是BD合并MDS最具特点的临床表现,而针刺反应阳性率较低(13.3%)。MDS分型以骨髓增生异常综合征合并难治性贫血(MDS-RA)最为常见(64.1%)。染色体核型异常常见(86%),8号三体异常占44.2%,且易伴发消化道损伤(78.9%)。结论:BD和MDS有合并出现的风险,BD合并MDS具有一定的临床特点,中老年、消化道损伤、8号染色体三体异常可能是BD伴发MDS的危险因素。Objective: To study the clinical characteristics of Behcet′s disease (BD) associated with myelodysplastic syndrome (MDS) and reduce clinical misdiagnosis. Methods: MDS was diagnosed in two patients mimicking BD referred to Nanjing Drum Tower Hospital in 2017.A reference search on published similar cases in Chinese database and the PubMed was performed and analyzed. Results: There were 60 patients (25 males and 35 females) who were diagnosed as BD associated with MDS.The average age was (45.1±15.3) years old (range 15-77 years old).Most of the patients were 40 to 69 years old (63.3%).BD preceded MDS in 61.7% of the cases.Digestive tract injury,fever and cytopenia were the most characteristic clinical manifestations of BD associated with MDS while the positive rate of acupuncture reaction was low (13.3%).The most common subtype of MDS was myelodysplastic syndrome accompanied with refractory anemia (MDS-RA)(64.1%).86% of the patients were found abnormal chromosomal karyotype.Trisomy 8 was the most common abnormal karyotype (44.2%),and easily associated with digestive tract injury (78.9%). Conclusion: BD and MDS have the risk of merging,BD combined with MDS has certain clinical characteristics.Older age,digestive tract injury and trisomy 8 may be the risk factors for BD associated with MDS.

关 键 词：白塞病 骨髓增生异常综合征 临床特征 

分 类 号：R593.22[医药卫生—内科学]