The Liver Unit, Addenbrooke's Hospital, Cambridge University Hospitals National Health Service Foundation Trust, Cambridge, United Kingdom.
Department of Histopathology, Addenbrooke's Hospital, Cambridge University Hospitals National Health Service Foundation Trust, Cambridge, United Kingdom.
Liver Transpl. 2018 Feb;24(2):171-181. doi: 10.1002/lt.24979.
Common variable immunodeficiency (CVID) is the most common form of primary immunodeficiency characterized by antibody deficiency, recurrent bacterial infections, and autoimmunity. Advanced chronic liver disease occurs in a subset of patients with CVID and manifests with various histological features, such as nodular regenerative hyperplasia, inflammation, fibrosis, and cholangiopathy. We present a case series characterizing the outcomes in adult patients transplanted for primary CVID-related liver disease. We discuss the unique transplantation challenges faced in this primary immunodeficiency group including susceptibility to infections and early disease recurrence. There is a statistically significant decrease in 3-year and 5-year survival after liver transplantation in those with CVID-related liver disease (55% at 3 and 5 years) compared with all-comers (89% at 3 years, 81% at 5 years), prompting a need for discussion of suitability of transplantation in this group of patients as well as methods for reducing posttransplantation risk such as scrupulous search for infectious agents and reduction of immunosuppression. Liver Transplantation 24 171-181 2018 AASLD.
普通变异性免疫缺陷症（CVID）是最常见的原发性免疫缺陷症，其特征是抗体缺乏、反复细菌感染和自身免疫。CVID 患者中会出现亚组的晚期慢性肝病，并表现出各种组织学特征，如结节性再生性增生、炎症、纤维化和胆管病。我们提出了一系列病例系列，描述了因原发性 CVID 相关肝病而接受移植的成年患者的结局。我们讨论了在这群原发性免疫缺陷症患者中面临的独特的移植挑战，包括对感染的易感性和早期疾病复发。与所有患者相比（3 年时为 89%，5 年时为 81%），CVID 相关肝病患者的肝移植后 3 年和 5 年生存率明显下降（3 年时为 55%，5 年时为 55%），这促使我们需要讨论该组患者移植的适宜性，以及降低移植后风险的方法，如仔细寻找感染源和减少免疫抑制。肝脏移植 24 171-181 2018 AASLD。
