Ameratunga Rohan, Storey Peter, Barker Russell, Jordan Anthony, Koopmans Wikke, Woon See-Tarn
a Department of Virology and Immunology , Auckland Hospital , Auckland , New Zealand.
b Clinical Immunology , Auckland Hospital , Auckland , New Zealand.
Expert Rev Clin Immunol. 2016;12(3):257-66. doi: 10.1586/1744666X.2016.1126509. Epub 2016 Feb 15.
Common variable immunodeficiency disorder (CVID) is the most frequent symptomatic primary immune deficiency disorder in adults. It probably comprises a spectrum of polygenic disorders, with hypogammaglobulinemia being the overarching feature. While the majority of patients with CVID can be identified with relative ease, a significant proportion can present with minimal symptoms in spite of profound laboratory abnormalities. Here we discuss three patients who were presented to the Auckland Hospital immunoglobulin treatment committee to determine if they qualified for immunoglobulin replacement. Two were asymptomatic with profound laboratory abnormalities while the third patient was severely ill with extensive bronchiectasis. The third patient had less severe laboratory abnormalities compared with the two asymptomatic patients. We have applied four sets of published diagnostic and treatment criteria to these patients to compare their clinical utility. We have chosen these patients from the broad phenotypic spectrum of CVID, as this often illustrates differences in diagnostic and treatment criteria.
普通可变免疫缺陷病（CVID）是成人中最常见的有症状的原发性免疫缺陷病。它可能包含一系列多基因疾病，低丙种球蛋白血症是其主要特征。虽然大多数CVID患者相对容易识别，但仍有相当一部分患者尽管实验室检查有严重异常，症状却很轻微。在此，我们讨论三名被提交至奥克兰医院免疫球蛋白治疗委员会以确定是否符合免疫球蛋白替代治疗条件的患者。其中两名患者无症状但实验室检查有严重异常，而第三名患者患有广泛支气管扩张且病情严重。与两名无症状患者相比，第三名患者的实验室异常情况没那么严重。我们将四套已发表的诊断和治疗标准应用于这些患者，以比较其临床实用性。我们从CVID广泛的表型谱中选取了这些患者，因为这常常能体现出诊断和治疗标准的差异。
