Kesh Susamita, Singh Umesh, Bernstein Jonathan A
From the Division of Immunology and Allergy, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
Division of Immunology/Allergy Section, Department of Internal Medicine, University of Cincinnati College of Medicine, Cincinnati, Ohio; and.
Allergy Asthma Proc. 2022 Sep 1;43(5):413-418. doi: 10.2500/aap.2022.43.220043.
Acquired angioedema (AAE) is a rare form of angioedema (AE) and is often associated with lymphoproliferative conditions and/or anti-C1 esterase inhibitor (C1-INH) antibodies without clear treatment consensus. Current treatments have been reported to have variable effectiveness with different safety concerns. A large Italian cohort of patients with AAE was previously found to respond well to tranexamic acid (TXA). Herein, we report our experience treating AAE with TXA used as prophylaxis. The objective was to describe clinical characteristics of patients with AAE and to report our experience with treating AAE with TXA. A retrospective chart review of patients with AAE (N = 13) from a large practice was conducted to assess characteristics and treatment responses. Patient demographics in addition to C1-INH quantitative, C1-INH functional, C4, and C1q levels; the presence of C1-INH antibodies; and a history of lymphoproliferative disease were extracted. The patients were also characterized by their treatment response to TXA. All the patients were white, with a mean age at diagnosis of 67 years, an average body mass index of 31.3 kg/m², and a male-to-female ratio of 7:6. Nine patients had positive C1-INH antibodies. The patients were on various prophylaxis treatments before TXA, including chemotherapy that targeted malignancy, cyclophosphamide, rituximab, and plasmapheresis. Ultimately, 11 of the 13 patients were on TXA for prophylaxis. At 1, 12, and 24 months after TXA treatment, attacks decreased by 97, 86, and 99%, respectively. One patient developed a deep vein thrombosis and TXA was stopped. These findings demonstrated that treatment of AAE with TXA was effective as prophylaxis for AE attacks. However, potential adverse effects remain a concern, which emphasizes the need for additional options.
获得性血管性水肿（AAE）是血管性水肿（AE）的一种罕见形式，常与淋巴增殖性疾病和/或抗C1酯酶抑制剂（C1-INH）抗体相关，目前尚无明确的治疗共识。据报道，目前的治疗方法疗效各异，且存在不同的安全问题。此前发现，一大组意大利AAE患者对氨甲环酸（TXA）反应良好。在此，我们报告使用TXA进行预防治疗AAE的经验。目的是描述AAE患者的临床特征，并报告使用TXA治疗AAE的经验。我们对一家大型医疗机构的AAE患者（N = 13）进行了回顾性病历审查，以评估其特征和治疗反应。提取了患者的人口统计学信息，以及C1-INH定量、C1-INH功能、C4和C1q水平；C1-INH抗体的存在情况；以及淋巴增殖性疾病史。还根据患者对TXA的治疗反应对其进行了特征描述。所有患者均为白人，诊断时的平均年龄为67岁，平均体重指数为31.3 kg/m²，男女比例为7:6。9例患者C1-INH抗体呈阳性。在使用TXA之前，患者接受了各种预防性治疗，包括针对恶性肿瘤的化疗、环磷酰胺、利妥昔单抗和血浆置换。最终，13例患者中有11例使用TXA进行预防。在TXA治疗后的1个月、12个月和24个月，发作次数分别减少了97%、86%和99%。1例患者发生了深静脉血栓形成，因此停用了TXA。这些发现表明，使用TXA治疗AAE作为预防AE发作是有效的。然而，潜在的不良反应仍然令人担忧，这凸显了需要更多治疗选择的必要性。
