Vecchi V, Burnelli R, Pileri S, Rosito P, Sabattini E, Civino A, Pericoli R, Paolucci G
Third Department of Pediatrics, University of Bologna, Italy.
Med Pediatr Oncol. 1993;21(6):402-10. doi: 10.1002/mpo.2950210603.
In 1985, Stein et al. (Blood 66:848-858) described a large cell lymphoma consisting of activated lymphoid elements, expressing the Reed-Sternberg cell-associated antigen Ki-1 (CD30); this tumor has recently been included in the updated Kiel classification and has been termed anaplastic large cell lymphoma (ALCL). This report concerns 13 patients with previously untreated ALCL who were admitted to the Pediatric Hematology and Oncology Department of Bologna University between January 1983 and December 1989. Ten cases were diagnosed as common type ALCL, 1 as Hodgkin's-related ALCL and the remaining 2 as histiocyte-rich ALCL/lymphohistiocytic T-cell lymphoma. T-cell markers were present in 85% of the cases, and B phenotype in the remaining 15%. About one-half of the patients presented systemic symptoms; all but one showed more than one involved site, mostly an association of nodal and extranodal sites. All patients (1 in stage I, 7 in stage II, and 5 in stage III) were treated with a modified version of the LSA2-L2 protocol. Survival and event-free survival at 4 years were 100% and 62.9%, respectively. Two out of 3 relapsed patients presented a recurrence of disease when they were off-therapy 24 and 36 months after first complete remission. Good response to salvage chemotherapy of all the relapsed patients and the long duration of the second complete remission were a distinctive characteristic of this neoplasm when compared to other histological subtypes. The clinical presentation, the tendency to develop late relapses and to achieve and maintain second remission easily suggest that ALCL is a high grade non-Hodgkin's lymphoma, from the histological point of view, but is similar to Hodgkin's disease from the clinical point of view. This would confirm the hypothesis that Hodgkin's disease and ALCL represent a continuous spectrum of the same disease.
1985年，斯坦因等人（《血液》66:848 - 858）描述了一种由活化淋巴细胞成分组成的大细胞淋巴瘤，表达与里德 - 斯腾伯格细胞相关的抗原Ki - 1（CD30）；这种肿瘤最近被纳入更新后的基尔分类，并被称为间变性大细胞淋巴瘤（ALCL）。本报告涉及1983年1月至1989年12月间收治于博洛尼亚大学儿科血液学和肿瘤学系的13例未经治疗的ALCL患者。10例被诊断为普通型ALCL，1例为霍奇金相关型ALCL，其余2例为组织细胞丰富型ALCL/淋巴组织细胞性T细胞淋巴瘤。85%的病例存在T细胞标志物，其余15%为B表型。约一半患者出现全身症状；除1例患者外，所有患者均有不止一个受累部位，多数为淋巴结和结外部位联合受累。所有患者（1例为Ⅰ期，7例为Ⅱ期，5例为Ⅲ期）均接受了改良版的LSA2 - L2方案治疗。4年时的生存率和无事件生存率分别为100%和62.9%。3例复发患者中有2例在首次完全缓解后24个月和36个月停止治疗时疾病复发。与其他组织学亚型相比，所有复发患者对挽救性化疗反应良好且第二次完全缓解持续时间长是该肿瘤的一个显著特征。从组织学角度看，ALCL的临床表现、晚期复发倾向以及易于获得和维持第二次缓解表明它是一种高级别非霍奇金淋巴瘤，但从临床角度看它与霍奇金病相似。这将证实霍奇金病和ALCL代表同一种疾病的连续谱这一假说。
