Wong Melanie
Department of Allergy and Immunology, The Children's Hospital at Westmead, Sydney, New South Wales, Australia.
J Paediatr Child Health. 2015 Feb;51(2):135-9. doi: 10.1111/jpc.12834.
Fifty years ago, in 1964, our understanding of the immune system was very rudimentary. Gell and Coombs had just described classes of hypersensitivity reactions, and Bruton had described and commenced immunoglobulin replacement in agammaglobulinaemia. The distinction between T and B cells was not identified and characterised until the 1960s and 1970s. This was followed by increasing recognition of T and B cell collaboration in immune responses and identification of significant immunodeficiencies. CD4 and CD8 T cells were only recognised in the 1970s and 1980s. We now know of five CD4 subsets; dysfunction of each is associated with different disorders. By 2014, advances in technology have enabled identification of the genetic basis of over 240 primary immunodeficiencies. Research into the gut microbiome and vitamin D holds promise for the understanding, treatment and prevention of autoimmune and allergic diseases. Immunoglobulin preparations for the treatment of antibody deficiencies improved with the development of preparations for intravenous then subcutaneous administration, giving patients choice and the ability for home-based treatment, especially if experiencing infusion associated adverse effects. Newborn screening for severe combined immunodeficiency is a reality. Improvements in haemopoietic stem cell transplantation and now gene therapy, albeit still only available in the research setting, are improving long-term survival in primary immunodeficiencies. Biologic therapeutic agents are improving the control of autoimmune disease but potentially leading to secondary immunodeficiency, increasing the risk of opportunistic infection and malignancy. It is an exciting time.
五十年前,即1964年,我们对免疫系统的了解还非常初级。盖尔和库姆斯刚刚描述了超敏反应的类型,布鲁顿描述了无丙种球蛋白血症并开始进行免疫球蛋白替代治疗。直到20世纪60年代和70年代,T细胞和B细胞的区别才被识别和描述。随后,人们越来越认识到T细胞和B细胞在免疫反应中的协作以及重要免疫缺陷的识别。CD4和CD8 T细胞直到20世纪70年代和80年代才被识别。我们现在知道有五个CD4亚群;每个亚群的功能障碍都与不同的疾病有关。到2014年,技术的进步使得超过240种原发性免疫缺陷的遗传基础得以识别。对肠道微生物群和维生素D的研究有望促进对自身免疫性疾病和过敏性疾病的理解、治疗和预防。随着静脉注射然后皮下注射制剂的发展,用于治疗抗体缺陷的免疫球蛋白制剂得到了改进,为患者提供了选择以及在家治疗的能力,特别是对于那些经历输液相关不良反应的患者。对严重联合免疫缺陷进行新生儿筛查已成为现实。造血干细胞移植以及现在的基因治疗(尽管仍仅在研究环境中可用)的改进正在提高原发性免疫缺陷患者的长期生存率。生物治疗药物正在改善自身免疫性疾病的控制,但可能导致继发性免疫缺陷,增加机会性感染和恶性肿瘤的风险。这是一个令人兴奋的时代。