凝血因子 XIII 缺乏症的诊治进展

陈 珏材; 杜 小清; 吴 红卫

陈珏材成都医学院第一附属医院
杜小清成都医学院第一附属医院
吴红卫成都医学院第一附属医院

关键词: 凝血功能障碍；FXIII 缺乏；遗传性；获得性

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。

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