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凝血因子 XIII 缺乏症的诊治进展

陈珏 材(, 杜小 清(, 吴红 卫(

摘要


凝血因子 XIII(FXIII)缺乏是一种罕见的出血疾病,主要表现为伤口愈合延迟、反复皮下血肿、
自发性流产、脐带出血以及颅内出血等。FXIII 缺乏分为遗传性或获得性,遗传性 FXIII 缺乏与基因突变相关,
获得性 FXIII 缺乏主要包括免疫介导以及非免疫性 FXIII 过度消耗或合成功能低下所致。识别 FXIII 缺乏及其潜
在原因是必要的,目前治疗手段包括血浆置换、丙种球蛋白、补充凝血因子 XIII、免疫抑制剂如糖皮质激素、
环磷酰胺以及利妥昔单抗等。本综述将针对凝血因子 XIII 缺乏的病因、诊断、临床特征和治疗选择进行梳理,
提高临床医生对本病的认识。

关键词


凝血功能障碍;FXIII 缺乏;遗传性;获得性



参考


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