Research progress of hemophagocytic lymphohistiocytosis associated with inflammatory bowel diseases
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摘要: 炎症性肠病(IBD)是噬血细胞综合征(HLH)发生的危险因素。病因分型中，IBD可以与HLH同属一种原发性免疫缺陷疾病，如2型X连锁淋巴组织细胞异常增生症(XLP-2)，也可以是继发性HLH的触发因素。目前有关IBD相关HLH的病例逐渐增多，这类情况下出现的HLH与IBD的诊疗非常复杂，因为一开始出现的HLH症状可能被认为是感染或是IBD疾病本身的恶化，疾病的延迟诊断会导致治疗不及时，最终可能解释HLH的高病死率。因此，这文对IBD相关HLH的研究进展进行综述，详细对此类人群的发病特征、机制、治疗及预后等方面进行总结分析，旨在为IBD合并HLH的诊疗管理提供思路与线索。
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关键词:
- 炎症性肠病 /
- 噬血细胞综合征 /
- 2型X连锁淋巴组织细胞异常增生症 /
- 克罗恩病 /
- 溃疡性结肠炎
Abstract: Inflammatory bowel diseases(IBD) is a risk factor for hemophagocytic lymphohistiocytosis(HLH). In the etiological classification, either IBD/HLH can be the same primary immunodeficiency disease, such as X-linked lymphoproliferative disease 2(XLP-2), or IBD is the trigger of secondary HLH. At present, the number of cases related to IBD associated with HLH is increasing gradually. The diagnosis and treatment of HLH and IBD in such cases is very complicated, because the initial symptoms of HLH may be considered as infection or the deterioration of the IBD itself. The delayed diagnosis of the disease leads to the delayed treatment, which may ultimately explain the high mortality rate of HLH. Therefore, this paper reviews the research progress of IBD-related HLH, and summarizes and analyzes the pathogenesis, mechanism, treatment and prognosis of this population in detail, aiming to provide ideas and clues for the diagnosis and treatment management of IBD associated with HLH. -
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