Department of Pediatrics, Oncology and Hematology, Medical University of Lodz, Lodz, Poland.
Mutat Res Rev Mutat Res. 2021 Jul-Dec;788:108386. doi: 10.1016/j.mrrev.2021.108386. Epub 2021 Jun 7.
Approximately 10 % of malignancies occur in carriers of germline mutations predisposing to cancer. A high risk of developing lymphomas has been noted in many primary immunodeficiencies, including DNA repair disorders. Moreover, implementation of next-generation sequencing has recently enabled to uncover rare genetic variants predisposing patients to lymphoid neoplasms. Some patients harboring inherited predisposition to lymphomas require dedicated clinical management, which will contribute to effective cancer treatment and to the prevention of potential severe toxicities and secondary malignancies. In line with that, our review summarizes the natural history of lymphoid tumors developing on different germline genetic backgrounds and discusses the progress that has been made toward successfully treating these malignancies.
约 10%的恶性肿瘤发生在易患癌症的种系突变携带者中。许多原发性免疫缺陷病,包括 DNA 修复障碍,都注意到发生淋巴瘤的风险很高。此外,下一代测序的实施最近使人们能够发现导致淋巴细胞肿瘤的罕见遗传变异。一些患有遗传性淋巴瘤倾向的患者需要专门的临床管理,这将有助于有效的癌症治疗和预防潜在的严重毒性和继发性恶性肿瘤。因此,我们的综述总结了在不同种系遗传背景下发展的淋巴肿瘤的自然史,并讨论了在成功治疗这些恶性肿瘤方面取得的进展。