Anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL) is an aggressive T-cell lymphoma characterized by large T-cells with strong CD30 and ALK expression. Although conventional chemotherapy is effective in most patients, approximately 30% experience a relapse or refractory disease and have a poor prognosis. Several risk factors associated with poor prognosis have been identified in pediatric ALK-positive ALCL. These include morphological patterns with the small cell variant or lymphohistiocytic variant, leukemic presentation, the presence of minimal disseminated disease, or involvement of the central nervous system. Relapsed or refractory ALK-positive ALCL is often resistant to conventional chemotherapy; therefore, salvage therapy is required. In recent years, targeted therapies such as ALK inhibitors and brentuximab vedotin (BV) have been developed. ALK inhibitors block the continuous activation of ALK kinase, a driver mutation that leads to cell proliferation in ALK-positive ALCL. Additionally, BV is an antibody–drug conjugate that targets CD30-positive cells. Both ALK inhibitors and BV have displayed dramatic effects in chemoresistant ALK-positive ALCL. Weekly vinblastine treatment and hematopoietic stem cell transplantation have also been reported to be effective therapies. This article reviews pediatric ALK-positive ALCL, focusing on risk factors and treatment strategies for pediatric patients with relapsed or refractory ALK-positive ALCL.
间变性淋巴瘤激酶（ALK）阳性间变性大细胞淋巴瘤（ALCL）是一种侵袭性T细胞淋巴瘤，其特征为具有强CD30和ALK表达的大T细胞。尽管常规化疗对大多数患者有效，但约30%的患者会出现复发或难治性疾病，预后较差。在儿童ALK阳性ALCL中，已发现多种与不良预后相关的风险因素，包括小细胞变异型或淋巴组织细胞变异型的形态学模式、白血病表现、存在微小播散性疾病或中枢神经系统受累。复发或难治性ALK阳性ALCL通常对常规化疗耐药，因此需要挽救治疗。近年来，靶向治疗如ALK抑制剂和维布妥昔单抗（BV）已得到开发。ALK抑制剂可阻断ALK激酶的持续激活，该驱动突变导致ALK阳性ALCL中的细胞增殖。此外，BV是一种靶向CD30阳性细胞的抗体-药物偶联物。ALK抑制剂和BV均在化疗耐药的ALK阳性ALCL中显示出显著疗效。每周长春碱治疗和造血干细胞移植也被报道为有效疗法。本文综述儿童ALK阳性ALCL，重点关注复发或难治性ALK阳性ALCL患儿的风险因素及治疗策略。
肿瘤(癌症)患者之家