Primary immunodeficiency disorders (PIDs) convey increased susceptibility to infections and sometimes to malignancies, particularly lymphomas. Such cancer development can be contributed by immune impairments resulting in weakened immunological surveillance against (pre)malignant cells and oncogenic viruses. Molecular defects in PID-patients are therefore being clarified, identifying new targets for innovative immunotherapy. Particularly pediatric cancers are being scrutinized, where over one-third of cancer-related deaths are accounted for by leukemia and lymphomas. Here we review how immunopathogenic mechanisms of several PIDs might associate with lymphoma development. We furthermore delineate existing immunotherapy strategies in adults for potential therapeutic application in childhood leukemia and lymphomas.

原发性免疫缺陷疾病（PID）传达了对感染以及有时对恶性肿瘤（尤其是淋巴瘤）的敏感性增加。这种癌症的发展可能是由免疫损伤引起的，导致针对（恶性）恶性细胞和致癌病毒的免疫学监测减弱。因此，PID患者的分子缺陷正在得到澄清，从而确定了创新免疫疗法的新目标。尤其是儿科癌症正在接受检查，其中三分之一以上的癌症相关死亡是由白血病和淋巴瘤引起的。在这里，我们回顾了几种PID的免疫致病机制可能与淋巴瘤的发展有关。我们还描述了成人中现有的免疫疗法策略，可用于儿童白血病和淋巴瘤的潜在治疗应用。