Many primary immunodeficiencies (PIDs) are recognised as being associated with malignancies, particularly lymphoid malignancies, which represent the highest proportion of cancers occurring in conjunction with this underlying condition. When patients present with genetic errors of immunity, clinicians must often reflect on whether to manage antitumoral treatment conventionally or to take a more personalised approach, considering possible existing comorbidities and the underlying status of immunodeficiency. Recent advances in antitumoral immunotherapies, such as monoclonal antibodies, antigen-specific adoptive cell therapies or compounds with targeted effects, potentially offer significant opportunities for optimising treatment for those patients, especially with lymphoid malignancies. In cases involving PIDs, variable oncogenic mechanisms exist, and opportunities for antitumoral immunotherapies can be considered accordingly. In cases involving a DNA repair defect or genetic instability, monoclonal antibodies can be proposed instead of chemotherapy to avoid severe toxicity. Malignancies secondary to uncontrolled virus-driven proliferation or the loss of antitumoral immunosurveillance may benefit from antivirus cell therapies or allogeneic stem cell transplantation in order to restore the immune antitumoral caretaker function. A subset of PIDs is caused by gene defects affecting targetable signalling pathways directly involved in the oncogenic process, such as the constitutive activation of phosphoinositol 3-kinase/protein kinase B (PI3K/AKT) in activated phosphoinositide 3-kinase delta syndrome (APDS), which can be settled with PI3K/AKT inhibitors. Therefore, immunotherapy provides clinicians with interesting antitumoral therapeutic weapons to treat malignancies when there is an underlying PID.

中文翻译：

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对与癌症相关的原发性免疫缺陷儿童进行免疫治疗的可能性。

许多原发性免疫缺陷（PID）被认为与恶性肿瘤有关，尤其是淋巴样恶性肿瘤，代表与这种潜在疾病相关的癌症发生率最高。当患者出现免疫遗传性错误时，临床医生必须经常考虑是否可能进行常规抗肿瘤治疗或采取更具个性化的方法，同时考虑可能存在的合并症和免疫缺陷的基本状况。抗肿瘤免疫疗法的最新进展，例如单克隆抗体，抗原特异性过继细胞疗法或具有靶向作用的化合物，可能为优化那些患者（尤其是淋巴恶性肿瘤）的治疗提供重要机会。在涉及PID的情况下，存在多种致癌机制，因此可以考虑抗肿瘤免疫疗法的机会。在涉及DNA修复缺陷或遗传不稳定的情况下，可以建议使用单克隆抗体代替化学疗法，以避免严重的毒性。继发于不受控制的病毒驱动的增殖或抗肿瘤免疫监视丧失的恶性肿瘤可受益于抗病毒细胞疗法或同种异体干细胞移植，以恢复免疫抗肿瘤看护人的功能。PID的子集是由影响直接参与致癌过程的可靶向信号通路的基因缺陷引起的，例如在活化的磷酸肌醇3激酶δ综合征（APDS）中磷酸肌醇3激酶/蛋白激酶B（PI3K / AKT）的组成性激活。 ，可以使用PI3K / AKT抑制剂解决。因此，