Salavoura Katerina, Kolialexi Aggeliki, Tsangaris George, Mavrou Ariadni
Medical Genetics, Athens University School of Medicine, Athens, Greece.
Anticancer Res. 2008 Mar-Apr;28(2B):1263-9.
Primary immunodeficiencies (PIDs) are genetic disorders that predispose to frequent and severe infections, autoimmunity and cancer. The expanded life span of such patients increases the overall risk for developing cancer, which is now estimated at 4-25%. The type of malignancy depends on the primary immunodeficiency, the age of the patient and possible viral infection, suggesting that different pathogenetic mechanisms are implicated in each case. Non-Hodgkin's lymphomas predominate, accounting for 60% of cases. The PIDs known to be associated with increased incidence of malignancy are: common variable immunodeficiency, IgA deficiency and DNA repair disorders. During recent years other types have also been included, such as severe combined immunodeficiency (SCID) and Wiskott Aldrich syndrome (WAS).
原发性免疫缺陷病（PIDs）是一类遗传性疾病，易导致频繁且严重的感染、自身免疫性疾病和癌症。这类患者寿命的延长增加了患癌的总体风险，目前估计该风险为4%-25%。恶性肿瘤的类型取决于原发性免疫缺陷、患者年龄以及可能的病毒感染，这表明每种情况涉及不同的致病机制。非霍奇金淋巴瘤最为常见，占病例的60%。已知与恶性肿瘤发病率增加相关的原发性免疫缺陷病有：常见变异型免疫缺陷、IgA缺乏症和DNA修复障碍。近年来，其他类型也被纳入其中，如重症联合免疫缺陷病（SCID）和威斯科特·奥尔德里奇综合征（WAS）。
