Diagnosis and management of ALK-positive anaplastic large cell lymphoma in children and adolescents

IF 2.2 4区医学 Q3 HEMATOLOGY Best Practice & Research Clinical Haematology Pub Date : 2023-03-01 DOI:10.1016/j.beha.2023.101444

Charlotte Rigaud , Fabian Knörr , Laurence Brugières , Wilhelm Woessmann

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引用次数: 4

Abstract

Anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL) is a CD30-positive T cell lymphoma characterized by signalling from constitutively activated ALK fusion proteins. Most children and adolescents present in advanced stages, often with extranodal disease and B symptoms. The current front-line therapy standard of six cycles polychemotherapy reaches an event-free survival of 70%. The strongest independent prognostic factors are minimal disseminated disease and early minimal residual disease. At relapse, ALK-inhibitors, Brentuximab Vedotin, Vinblastine, or second line chemotherapy are effective re-inductions. Survival at relapse exceeds 60–70% with consolidation according to the time of relapse (Vinblastine monotherapy or allogeneic hematopoietic stem cell transplantation) so that the overall survival reaches 95%. It needs to be shown whether check-point inhibitors or long-term ALK-inhibition may substitute for transplantation. The future necessitates international cooperative trials testing whether a shift of paradigm to a chemotherapy-free regimen can cure ALK-positive ALCL.

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儿童和青少年alk阳性间变性大细胞淋巴瘤的诊断和治疗

间变性淋巴瘤激酶（ALK）阳性的间变性大细胞淋巴瘤（ALCL）是一种CD30阳性的T细胞淋巴瘤，其特征是来自组成型激活的ALK融合蛋白的信号传导。大多数儿童和青少年处于晚期，通常伴有结外疾病和B症状。目前的一线治疗标准是六个周期的综合化疗，无事件生存率达到70%。最有力的独立预后因素是最小播散性疾病和早期最小残留性疾病。复发时，ALK抑制剂、布仑妥昔单抗韦多汀、长春碱或二线化疗是有效的再诱导。复发时的生存率超过60-70%，根据复发时间巩固（长春碱单药治疗或异基因造血干细胞移植），因此总生存率达到95%。需要证明检查点抑制剂或长期ALK抑制是否可以替代移植。未来需要进行国际合作试验，测试向无化疗方案转变的模式是否可以治愈ALK阳性ALCL。

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

Best Practice & Research Clinical Haematology 医学-血液学

CiteScore

4.20

自引率

0.00%

发文量

审稿时长

35 days

期刊介绍： Best Practice & Research Clinical Haematology publishes review articles integrating the results from the latest original research articles into practical, evidence-based review articles. These articles seek to address the key clinical issues of diagnosis, treatment and patient management. Each issue follows a problem-orientated approach which focuses on the key questions to be addressed, clearly defining what is known and not known, covering the spectrum of clinical and laboratory haematological practice and research. Although most reviews are invited, the Editor welcomes suggestions from potential authors.