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目的:探讨噬血细胞综合征弥散性血管内凝血(DIC)的特点及治疗体会。方法:回顾性总结了20例在我院住院的噬血细胞综合征并发DIC的临床特点及治疗上的体会。结果:发现噬血细胞综合征起病急骤、病情变化快,几乎在确诊噬血细胞综合征的同时DIC即已存在,与其他疾病的DIC相比,更常见,更凶险,治疗更棘手,除大出血外,顽固性多脏器功能衰竭多见,尤以肝衰最常见。治疗上除了DIC的常规治疗外,激素和免疫治疗应早期应用。而免疫治疗的早期应用又取决于噬血细胞综合征的早期诊断。结论:噬血细胞综合征DIC的特点是:DIC出现早,顽固性多脏器衰竭中以肝衰最常见。治疗上积极针对DIC治疗,为噬血细胞综合征免疫救治赢得了时间。噬血细胞综合征早期诊断及早期进行免疫治疗是DIC根本好转的关键。
Objective: To investigate the characteristics and treatment of diffuse intravascular coagulation (DIC) in hemophagocytic syndrome. Methods: The clinical features and treatment of 20 patients with hemophagocytic syndrome complicated with DIC hospitalized in our hospital were retrospectively reviewed. Results: Hemophagocytic syndrome was found to have a mild onset and a rapidly changing disease. DIC was already present almost at the time of diagnosis of hemophagocytic syndrome. Compared with DIC of other diseases, it is more common and more dangerous and the treatment is more difficult. In addition to hemorrhage , Intractable multiple organ failure more common, especially the most common liver failure. In addition to the treatment of conventional DIC treatment, hormone and immunotherapy should be used early. The early application of immunotherapy in turn depends on the early diagnosis of hemophagocytic syndrome. CONCLUSIONS: The characteristic of hemophagocytic syndrome DIC is that liver failure is most common in early and intractable multiple organ failure in DIC. Positive treatment for DIC treatment for immune recovery of hemophagocytic syndrome won the time. Early detection of hemophagocytic syndrome and early immunotherapy is the key to the fundamental improvement of DIC.