PDF(Pubmed)
Abstract：
Acquired angioedema (AAE) due to deficiency of a C1 esterase inhibitor (C1-INH; AAE-C1-INH) is a rare and potentially fatal syndrome characterized by recurrent episodes of angioedema without urticaria. Often underdiagnosed due to its rarity and mimicry of common allergic reactions, AAE-C1-INH is associated with lymphoproliferative disorders, necessitating early recognition for improved outcomes. We present a case of a 63-year-old male diagnosed with AAE-C1-INH and concurrent stage 0 chronic lymphocytic leukemia (CLL), a rarely documented association. Despite chemotherapy, the patient experienced persistent angioedema until C1 esterase inhibitor therapy was initiated. This case underscores the importance of screening for lymphoproliferative disorders in AAE-C1-INH patients and explores refractory cases, urging further research into mechanisms and treatment strategies.
摘要：
由于C1酯酶抑制剂（C1-INH;AAE-C1-INH）缺乏而导致的获得性血管性水肿（AAE）是一种罕见且可能致命的综合征，其特征是血管性水肿反复发作而没有荨麻疹。由于其稀有性和对常见过敏反应的模仿，通常未被诊断，AAE-C1-INH与淋巴增生性疾病有关，需要及早认识到改善的结果。我们介绍了一例63岁的男性，诊断为AAE-C1-INH和并发0期慢性淋巴细胞白血病（CLL），很少有记录的协会。尽管化疗,患者出现持续性血管性水肿,直至开始C1酯酶抑制剂治疗.该病例强调了在AAE-C1-INH患者中筛查淋巴增生性疾病的重要性，并探讨了难治性病例，敦促进一步研究机制和治疗策略。
