炎症性肠病合并骨髓增生异常综合征9例临床分析并文献复习

Analysis of 9 patients of inflammatory bowel disease concurrence with myelodysplastic syndrome and review of the Literature

目的通过对炎症性肠病伴发骨髓增生异常综合征患者的临床资料进行分析,探寻两种疾病间内在联系。方法对我院1985-2006年炎症性肠病伴发骨髓增生异常综合征患者9例的临床资料进行分析并复习相关文献报道。方法我院炎症性肠病合并MDS患者中以克罗恩病为主,占77%(7/9)。我院患者MDS多先发与IBD,IBD发病年龄为(38.7±10.4)岁,MDS发病年龄为(38.3±9.7)岁。经过治疗后部分IBD可获得缓解,但对于MDS的病情无明显影响。国外报道多以IBD首发多见,以克罗恩病多见,IBD平均发病年龄(57.62±17.58)岁,MDS平均发病年龄为(63.46±15.51)岁。国外病例发现存在染色体异常患者18例,无染色体异常者23例,存在染色体异常的患者无论是MDS或是IBD的发病年龄均较无染色体异常的患者为大,有染色体异常的患者两病发病间隔时间明显短于无染色体异常的患者。结论两病伴发患者的发病年龄与疾病单发时的高发年龄有明显不同,提示两种疾病间存在关联及相互作用。两病伴发的机制考虑与免疫异常、染色体异常相关。治疗上针对IBD的治疗一般可以控制IBD的病情,但对于MDS的病情未见明确影响。骨髓移植可能作为同时治疗两种疾病的选择。

[ Objective ] To study the relationship between inflammatory bowel disease （IBD） and myelodysplastie syndrome （MDS）, we characterized the clinical features of patients with IBD and MDS in our hospital. [ Methods ] We analyze the clinical features of 9 cases with both MDS and IBD hospitalized in our hospital from 1985-2006. We reviewed pertinent literatures in recent years and summarize the feature of IBD and MDS concurrence. [ Results ] We found crohn＇s disease in seven cases, ulcerative colitis in two case. MDS was diagnosed first in most cases. Average onset age of IBD is （38.7±10.4） years; average onset age of MDS is （38.3±9.7） year. Some case＇s state of IBD got controlled after proper management but the state of MDS remains. The data we summarized from pertinent literatures showed that average age of onset of IBD is （57.62±17.58） year, average age of onset of MDS is （63.46±15.51） year; eases with chromosomal abnormality have an older average onset age and shorter interval time than cases with out chromosomal abnormality; some case＇s state of IBD got controlled after proper management but the state of MDS remains, which is similar to our cases. [ Conclusion ] There are obvious difference in onset age in two disease concurrent compared with those who two disease onset separately, this indicated there are some relations and interactions between IBD and MDS. There are mechanisms alike such as chromosomal abnormality immunologic abnormality lie in bot：h diseases. Therapy can control state of IBD, but showed no effect on the state of MDS. BMT may be a new choice for cased with IBD and MDS.