Background: Acquired angioedema due to C1 inhibitor deficiency (AAE-C1-INH) is a very rare disease. In clinical practice, it may be difficult to differentiate AAE-C1-INH from hereditary angioedema due to C1-INH deficiency (HAE-C1-INH). In both conditions, patients are at an increased risk of death from asphyxiation due to upper airway obstruction. The association of AAE-C1-INH with lymphoproliferative and autoimmune diseases, and with presence of anti-C1-INH antibodies has been well documented, and treatment of the underlying condition may result in complete remission of angioedema. Objectives: To discuss the clinical evaluation, diagnosis, and treatment outcomes of AAE-C1-INH in the context of the care of 2 patients with recurrent isolated angioedema. Methods: Two patients were followed up prospectively at our clinic. Measurements of C3, C4, C1-INH, and C1q levels were carried out by nephelometry, and the functional activity of C1-INH was determined by a chromogenic assay. Hematological investigation included morphological and immunophenotyping analysis of peripheral blood, bone marrow, and spleen histopathology. Sequencing of the 8 exons and adjacent intronic regions of the SERPING1 gene was performed using the Sanger method. Results: Two patients were diagnosed with AAE-C1-INH associated with splenic marginal zone lymphoma during follow-up. Conclusions: Close follow-up, including detailed clinical history, physical examination, and laboratory tests, of our patients with AAE-C1-INH was essential for the early diagnosis and successful treatment of the lymphoproliferative disease, leading to the resolution of the angioedema attacks.
Int Arch Allergy Immunol

中文翻译：

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由于脾脏边缘区淋巴瘤发生之前C1抑制剂缺乏导致的获得性血管性水肿：临床实践的进一步见解。

背景：由于C1抑制剂缺乏（AAE-C1-INH）而获得的血管性水肿是一种非常罕见的疾病。在临床实践中，由于C1-INH缺乏症（HAE-C1-INH），可能难以将AAE-C1-INH与遗传性血管性水肿区分开。在这两种情况下，患者由于上呼吸道阻塞而窒息死亡的风险增加。AAE-C1-INH与淋巴增生性疾病和自身免疫性疾病以及存在抗C1-INH抗体的相关性已得到很好的证明，对潜在疾病的治疗可能导致血管性水肿的完全缓解。目的：探讨AAE-C1-INH在2例复发性孤立性血管性水肿患者的护理中的临床评估，诊断和治疗结果。方法：我们的诊所对两名患者进行了前瞻性随访。通过浊度法测量C3，C4，C1-INH和C1q水平，并通过生色测定法确定C1-INH的功能活性。血液学调查包括外周血，骨髓和脾组织病理学的形态学和免疫表型分析。使用Sanger方法对SERPING1基因的8个外显子和相邻的内含子区域进行测序。结果： 2例患者在随访期间被诊断出患有AAE-C1-INH合并脾边缘区淋巴瘤。结论：对我们的AAE-C1-INH患者进行密切的随访，包括详细的临床病史，体格检查和实验室检查，对于淋巴增生性疾病的早期诊断和成功治疗至关重要，从而可以解决血管性水肿的发作。
Int Arch过敏免疫