Pediatric Onco-Hematology Unit, University Hospital of Padova, Padova, Italy.
Pediatr Blood Cancer. 2012 Nov;59(5):828-33. doi: 10.1002/pbc.24125. Epub 2012 Mar 2.
Anaplastic large cell lymphoma (ALCL) represents approximately 15% of all pediatric non-Hodgkin lymphomas (NHL). It has distinct clinical features, including frequent involvement of extranodal sites and rare localization to the central nervous system (CNS). Despite varying treatment approaches the outcome of patients with ALCL has not significantly improved during the last two decades.
From October 1997 to beginning of 2000, newly diagnosed ALCL patients were enrolled into AIEOP LNH-97 protocol for ALCL. Thereafter and until 2007, only CNS positive patients were included. AIEOP LNH-97 was based on the BFM-95 schema for ALCL and included six high-dose chemotherapy courses. CNS prophylaxis was obtained with one intrathecal injection of chemotherapy in each course, whereas treatment of CNS involvement included three intrathecal injections without irradiation.
Thirty-two patients were eligible for the study. Lymph-node disease was the most frequent localization (69% of the cases), followed by mediastinal (25%), CNS (22%), bone marrow (16%), and skin (13%) involvement. Probabilities of overall survival (OS) and of event-free survival (EFS) at 5 years for the whole population were 87% (SE 6%) and 68% (SE 8%), respectively.
This study confirmed that short pulse chemotherapy is an efficacious treatment option for first line therapy of pediatric ALCL, and that dose intensity may have some relevance for outcome, but not in all of the patients. Refinement and optimization of therapy strategies for ALCL may originate from a combination of clinical and biological prospective studies, as those in the pipeline of current international collaboration.
间变大细胞淋巴瘤（ALCL）约占儿童非霍奇金淋巴瘤（NHL）的 15%。它具有独特的临床特征，包括经常涉及结外部位和罕见的中枢神经系统（CNS）定位。尽管治疗方法各异，但在过去二十年中，ALCL 患者的预后并未显著改善。
从 1997 年 10 月到 2000 年初，新诊断的 ALCL 患者被纳入 AIEOP LNH-97 方案治疗 ALCL。此后直至 2007 年，仅纳入 CNS 阳性患者。AIEOP LNH-97 基于 BFM-95 方案治疗 ALCL，包括六个高剂量化疗疗程。CNS 预防采用每个疗程一次鞘内注射化疗，而 CNS 受累的治疗包括三次鞘内注射而不进行放疗。
32 名患者符合研究条件。淋巴结疾病是最常见的定位（69%的病例），其次是纵隔（25%）、CNS（22%）、骨髓（16%）和皮肤（13%）受累。全人群的总生存率（OS）和无事件生存率（EFS）在 5 年时分别为 87%（SE 6%）和 68%（SE 8%）。
本研究证实，短脉冲化疗是儿科 ALCL 一线治疗的有效治疗选择，剂量强度可能与疗效相关，但并非对所有患者都相关。通过目前国际合作的临床试验，可能会对 ALCL 的治疗策略进行改进和优化。
