Hermans Cedric, Vander Vorst Sébastien, Lambert Catherine
Hemostasis and Thrombosis Unit, Division of Adult Hematology, Belgium.
Blood Coagul Fibrinolysis. 2012 Mar;23(2):155-7. doi: 10.1097/MBC.0b013e32834ee10d.
Hereditary angioedema (HAE) is a rare genetic disorder causing a deficiency in C1 esterase inhibitor (C1-INH) that is manifested through unpredictable oedema. We describe a case of a patient with HAE who had previously been refused surgery for tonsillitis due to the potential for oedema, in whom regular monitoring of C1-INH levels combined with intensified therapy with danazol, tranexamic acid and C1-INH concentrate enabled an uncomplicated procedure with no oedema crisis. However, clinicians should be alerted that higher overall dosages of C1-INH concentrate for perioperative prophylaxis may be required than those typically used to treat acute HAE attacks.
遗传性血管性水肿(HAE)是一种罕见的遗传性疾病,会导致C1酯酶抑制剂(C1-INH)缺乏,表现为不可预测的水肿。我们描述了一例HAE患者,该患者此前因存在水肿风险而被拒绝进行扁桃体炎手术,通过定期监测C1-INH水平,并联合使用达那唑、氨甲环酸和C1-INH浓缩物强化治疗,使其能够顺利进行手术,且未发生水肿危象。然而,临床医生应警惕,围手术期预防所需的C1-INH浓缩物总剂量可能高于通常用于治疗急性HAE发作的剂量。