原发性免疫缺陷疾病中血液系统恶性肿瘤风险增加：免疫治疗的机会。

Increased risk of hematologic malignancies in primary immunodeficiency disorders: opportunities for immunotherapy.

机构信息

Department of Pediatrics and Laboratory of Translational Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.

Princess Máxima Center for Pediatric Oncology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands.

出版信息

Clin Immunol. 2018 May;190:22-31. doi: 10.1016/j.clim.2018.02.007. Epub 2018 Feb 27.

DOI:10.1016/j.clim.2018.02.007

PMID:29499421

Abstract

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.

摘要

原发性免疫缺陷病（PID）使患者更容易感染，有时还会导致恶性肿瘤，尤其是淋巴瘤。这种癌症的发展可能是由于免疫功能受损导致的，使患者对（前）恶性细胞和致癌病毒的免疫监测减弱。因此，正在阐明 PID 患者的分子缺陷，以确定新的免疫治疗创新目标。特别是儿科癌症受到了密切关注，其中超过三分之一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 的免疫发病机制如何与淋巴瘤的发展相关联。我们还描述了成人中现有的免疫治疗策略，以潜在地应用于儿童白血病和淋巴瘤的治疗。