Primary immunodeficiency diseases associated with increased susceptibility to viral infections and malignancies
Primary immunodeficiency diseases associated with increased susceptibility to viral infections and malignancies
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DOI：
10.1016/j.jaci.2011.02.047
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发表时间：
2011-06-01
影响因子：
14.2
通讯作者：
Nichols, Kim E.
中科院分区：
文献类型：
--
作者：
Rezaei, Nima;Hedayat, Mona;Nichols, Kim E.
原发性免疫缺陷（PID）通常的特征是对特定感染的易感性增加，在某些情况下，恶性肿瘤的发病率高于正常。尽管PID的诊断和早期治疗的改善降低了感染的早期发病率和死亡率，但癌症的发展仍然是过早死亡的重要原因。PID患者出现癌症通常是由于免疫应答受损，导致对致癌病毒、癌前病变或恶性细胞或两者的监视减弱。在此，我们回顾了与病毒感染和癌症易感性增强相关的几种PID的临床和生物学特征，包括X连锁淋巴组织增生性疾病、IL-2诱导的T细胞激酶缺乏症、疣状表皮发育不良、疣、低丙种球蛋白血症、感染和骨髓增生综合征、常染色体隐性高IgE综合征、X连锁无丙种球蛋白血症和常见变异型免疫缺陷。重要的是，我们深入了解这些独特PID的基本分子性质，以更好地了解病毒相关恶性肿瘤的发病机制，并开发创新的治疗策略。（J Allergy Clin Immunol 2011;127：1329-41.）
Primary immunodeficiencies (PIDs) are commonly characterized by an increased susceptibility to specific infections and, in certain instances, a higher than usual incidence of malignancies. Although improved diagnosis and early treatment of PIDs have reduced early morbidity and mortality from infection, the development of cancer remains a significant cause of premature death. The emergence of cancer in patients with PIDs often results from impairments in the immune response that lead to weakened surveillance against oncogenic viruses, premalignant or malignant cells, or both. Here we review the clinical and biologic features of several PIDs associated with enhanced susceptibility to viral infections and cancer, including X-linked lymphoproliferative disease; IL-2-inducible T-cell kinase deficiency; epidermodysplasia verruciformis; warts, hypogammaglobulinemia, infections, and myelokathexis syndrome; autosomal recessive hyper-IgE syndrome; X-linked agammaglobulinemia; and common variable immunodeficiency. It is of importance that we gain in-depth insights into the fundamental molecular nature of these unique PIDs to better understand the pathogenesis of virus-associated malignancies and to develop innovative therapeutic strategies. (J Allergy Clin Immunol 2011;127:1329-41.)