Reiter A, Schrappe M, Tiemann M, Parwaresch R, Zimmermann M, Yakisan E, Dopfer R, Bucsky P, Mann G, Gadner H
Department of Pediatric Hematology and Oncology, Medizinische Hochschule, Hannover, Germany.
J Clin Oncol. 1994 May;12(5):899-908. doi: 10.1200/JCO.1994.12.5.899.
To prove prospectively the efficacy of a short-pulse chemotherapy for treatment of Ki-1 anaplastic large-cell lymphoma (ALCL) of childhood.
From October 1983 to December 1992, 62 patients (median age, 9.7 years) with newly diagnosed Ki-1 ALCL were enrolled onto Non-Hodgkin's Lymphoma-Berlin-Frankfurt-Munster (NHL-BFM) studies 83, 86, and 90. The most frequent immunophenotype was T cell. Ki-1 ALCL differed from other subsets of NHL of childhood by the more frequent involvement of bone, soft tissue, and skin, and by the lack of bone marrow (BM) disease. A 5-day prephase course (prednisone/cyclophosphamide) was followed by two different 5-day courses of chemotherapy: course A consisted of dexamethasone, methotrexate (MTX) 0.5 g/m2 (24-hour infusion), intrathecal chemotherapy, ifosfamide, cytarabine (Ara-C), and etoposide (VP-16); course B consisted of cyclophosphamide and doxorubicin instead of ifosfamide, and Ara-C/VP-16, respectively. Treatment was stratified into three branches. Branch 1 (stage I and stage II resected) received three courses; branch 2 (stage II not resected, stage III), six courses; and branch 3 (stage IV), six intensified courses containing MTX 5 g/m2, and Ara-C 2 g/m2. Local radiotherapy was not performed.
Four patients failed to enter remission, and one died of infection. Seven patients relapsed within 9 months after diagnosis; two patients had isolated local relapses, but BM and CNS were never involved. Fifty patients have been in first continuous complete remission (CR) for 0.6 to 9.7 years (median, 2.5), and 56 are alive. The probabilities for survival and event-free survival (EFS) at 9 years are 83% +/- 7% (SE) and 81% +/- 5%. Skin involvement was the only negative prognostic parameter.
Short-pulse chemotherapy over 2 to 5 months without local therapy modalities is effective in the treatment of Ki-1 ALCL.
前瞻性地证明短脉冲化疗治疗儿童Ki-1间变性大细胞淋巴瘤（ALCL）的疗效。
1983年10月至1992年12月，62例新诊断的Ki-1 ALCL患者（中位年龄9.7岁）入组非霍奇金淋巴瘤-柏林-法兰克福-明斯特（NHL-BFM）研究83、86和90。最常见的免疫表型为T细胞。Ki-1 ALCL与儿童NHL的其他亚组不同，其骨骼、软组织和皮肤受累更为频繁，且无骨髓（BM）疾病。先进行为期5天的前期疗程（泼尼松/环磷酰胺），随后进行两个不同的为期5天的化疗疗程：A疗程包括地塞米松、甲氨蝶呤（MTX）0.5 g/m²（24小时输注）、鞘内化疗、异环磷酰胺、阿糖胞苷（Ara-C）和依托泊苷（VP-16）；B疗程分别用环磷酰胺和多柔比星替代异环磷酰胺以及Ara-C/VP-16。治疗分为三个分支。分支1（I期和II期切除）接受三个疗程；分支2（II期未切除、III期）接受六个疗程；分支3（IV期）接受六个强化疗程，包括MTX 5 g/m²和Ara-C 2 g/m²。未进行局部放疗。
4例患者未进入缓解期，1例死于感染。7例患者在诊断后9个月内复发；2例患者为孤立性局部复发，但BM和中枢神经系统从未受累。50例患者首次持续完全缓解（CR）0.6至9.7年（中位值2.5年），56例患者存活。9年时的生存率和无事件生存率（EFS）分别为83%±7%（SE）和81%±5%。皮肤受累是唯一的不良预后参数。
在2至5个月内进行短脉冲化疗且不采用局部治疗方式，对Ki-1 ALCL的治疗有效。
