Chapel Helen, Cunningham-Rundles Charlotte
Department of Clinical Immunology, Oxford Radcliffe Hospitals, Oxford, UK.
Br J Haematol. 2009 Jun;145(6):709-27. doi: 10.1111/j.1365-2141.2009.07669.x. Epub 2009 Mar 30.
The common variable immunodeficiency disorders are a mixed group of heterogeneous conditions linked by lack of immunoglobulin production and primary antibody failure. This variability results in difficulty in making coherent sense of either immunopathogenesis or the role of various genetic abnormalities reported in the literature. The recent attempt to collate the varied complications in these conditions and to define particular clinical phenotypes has improved our understanding of these diseases. Once refined and confirmed by other studies, these definitions will facilitate improved accuracy of prognosis and better management of clinical complication. They may also provide a method of analysing outcomes as related to new immunopathological and genetic findings.
常见可变免疫缺陷病是一组混合性的异质性疾病,其共同特点是免疫球蛋白产生缺乏和原发性抗体缺陷。这种变异性导致难以对免疫发病机制或文献中报道的各种基因异常的作用形成连贯的认识。最近对这些疾病中各种并发症进行整理并定义特定临床表型的尝试,增进了我们对这些疾病的理解。一旦这些定义经过其他研究的完善和确认,将有助于提高预后的准确性,并更好地管理临床并发症。它们还可能提供一种分析与新的免疫病理学和基因发现相关的结果的方法。