Brouet J C, Seligmann M
Bull Cancer. 1978;65(1):89-94.
The occurrence of malignant proliferative diseases in patients with primary immune deficiencies is far more frequent than in normal individuals. These malignant proliferations may supervent in all types of immune deficiencies but are more frequent in the Wiskott-Aldrich syndrom, ataxia telangiectasia and variable immunodeficiencies. The incidence of malignant lymphomas is striking since they account for two thirds of the malignancies. This fact does not support the hypothesis of a faulty immunological surveillance and would be in accordance with the hypothesis of a direct role of antigenic stimulations occurring on an immune system devoid of regulatory mechanisms.
原发性免疫缺陷患者中恶性增殖性疾病的发生远比正常个体频繁。这些恶性增殖可能发生在所有类型的免疫缺陷中，但在维斯科特-奥尔德里奇综合征、共济失调毛细血管扩张症和可变免疫缺陷中更为常见。恶性淋巴瘤的发病率惊人，因为它们占恶性肿瘤的三分之二。这一事实不支持免疫监视缺陷的假说，而与在缺乏调节机制的免疫系统上发生的抗原刺激的直接作用假说相符。
