Treatment of lymphoid malignancies in patients with primary immunodeficiencies associated with DNA repair defects

Q4 Medicine
L. K. Anderzhanova, Y. Rodina, A. Mukhina, Y. Abugova, D. Abramov, M. Aleksenko, L. A. Vavilova, Y. Y. Dyakonova, D. Evstratov, E. Raykina, V. Fominykh, A. Shcherbina, E. Deripapa, N. Myakova
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引用次数: 0

Abstract

Nijmegen breakage syndrome (NBS) and ataxia-telangiectasia (AT; Louis–Bar syndrome) are primary immunodeficiencies (PID) associated with chromosome instability and DNA repair defects that predispose individuals to an increased risk of various malignancies. In our study, we retrospectively analyzed clinical characteristics and outcomes of 28 cancer cases in 14 patients with AT and 10 patients with NBS who had been treated at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology between January 2007 and December 2022. The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. The most common type of malignancy was mature B-cell non-Hodgkin lymphoma (B-NHL) (42%), with diffuse large B-cell lymphoma (DLBCL) accounting for 91% of all B-NHL cases. Other cases included T-cell acute lymphoblastic leukemia (ALL) (n = 3), B-cell ALL (n = 2), Hodgkin lymphoma (n = 3), NK/T-cell lymphoma (n = 1), T-cell lymphoblastic lymphoma (n = 1), peripheral T- cell lymphoma (n = 2), medulloblastoma (n = 1) epithelioid rhabdomyosarcoma (n = 1), T-cell prolymphocytic leukemia (n = 2). A total of 4 patients were diagnosed with second malignancies (2 children with AT and 2 children with NBS. The diagnosis of PID was suspected or confirmed before the initiation of cancer therapy in 62% of AT patients and in 100% of NBS patients. Treatment was given in accordance with standard protocols with chemotherapy dose modifications. A total of 93% of patients with AT and 80% of patients with NBS required dose reduction. The level of response was quite high: 81% of patients with AT and 58% of patients with NBS achieved complete remission. According to our data, the use of reduced-dose chemotherapy regimens helps to achieve an acceptable toxicity profile without reducing the overall effectiveness of treatment.
与DNA修复缺陷相关的原发性免疫缺陷患者淋巴细胞恶性肿瘤的治疗
奈亨断裂综合征(NBS)和共济失调-毛细血管扩张症(AT);Louis-Bar综合征是一种原发性免疫缺陷(PID),与染色体不稳定和DNA修复缺陷有关,使个体易患各种恶性肿瘤的风险增加。在我们的研究中,我们回顾性分析了2007年1月至2022年12月在Dmitry Rogachev国家儿童血液学、肿瘤学和免疫学医学研究中心接受治疗的14例AT患者和10例NBS患者的28例癌症的临床特征和结局。该研究得到了独立伦理委员会和Dmitry Rogachev国家儿童血液学、肿瘤学和免疫学医学研究中心科学委员会的批准。最常见的恶性肿瘤类型是成熟b细胞非霍奇金淋巴瘤(B-NHL)(42%),弥漫性大b细胞淋巴瘤(DLBCL)占所有B-NHL病例的91%。其他病例包括T细胞急性淋巴母细胞白血病(ALL) (n = 3)、b细胞ALL (n = 2)、霍奇金淋巴瘤(n = 3)、NK/T细胞淋巴瘤(n = 1)、T细胞淋巴母细胞淋巴瘤(n = 1)、外周T细胞淋巴瘤(n = 2)、成神经管细胞瘤(n = 1)、上皮样横纹肌肉瘤(n = 1)、T细胞前淋巴细胞白血病(n = 2)。共4例患者被诊断为第二恶性肿瘤(2例患儿为AT, 2例患儿为NBS)。62%的AT患者和100%的NBS患者在开始癌症治疗前怀疑或证实了PID的诊断。治疗按照化疗剂量调整的标准方案进行。总共93%的AT患者和80%的NBS患者需要减少剂量。反应水平相当高:81%的AT患者和58%的NBS患者达到完全缓解。根据我们的数据,使用小剂量化疗方案有助于达到可接受的毒性特征,而不会降低治疗的总体有效性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Pediatric Hematology/Oncology and Immunopathology
Pediatric Hematology/Oncology and Immunopathology Medicine-Pediatrics, Perinatology and Child Health
CiteScore
0.40
自引率
0.00%
发文量
49
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