NON-HODGKINS LYMPHOMAS OF CHILDHOOD - AN ANALYSIS OF THE HISTOLOGY, STAGING, AND RESPONSE TO TREATMENT OF 338 CASES AT A SINGLE INSTITUTION
NON-HODGKINS LYMPHOMAS OF CHILDHOOD - AN ANALYSIS OF THE HISTOLOGY, STAGING, AND RESPONSE TO TREATMENT OF 338 CASES AT A SINGLE INSTITUTION
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DOI：
10.1200/jco.1989.7.2.186
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发表时间：
1989-02-01
影响因子：
45.3
通讯作者：
BERARD, CW
中科院分区：
文献类型：
--
作者：
MURPHY, SB;FAIRCLOUGH, DL;BERARD, CW
在1962年至1986年期间，共有338名连续新诊断的儿童和青少年非霍奇金淋巴瘤（NHL）在圣裘德儿童研究医院（SJCRH）进行了评估和治疗。中位随访时间为6.6年（范围：1.8 - 23年）。患者年龄范围为7个月至21岁（中位数，10岁），71%为男性。所有病例均通过临床分期系统进行分期（I至IV）。18%为1期，21%为II期，43%为III期，18%为IV期。诊断时明确为白血病的病例（即，> 25%骨髓母细胞）从分析中排除。所有病例的病理材料均根据工作公式进行审查和分类。病例的组织学分布如下：38.8%弥漫性小无裂细胞（未分化、伯基特和非伯基特）; 26.3%弥漫性大细胞，主要为免疫母细胞; 28.1%淋巴母细胞; 6.8%其他。随着时间的推移，治疗策略逐渐发展为针对治疗分配的阶段和组织学特异性策略，总体结果从37%（. ±. 1975年以前治疗的患者的2年无事件生存率（EFS）为77%（± 5%）。4%，自1978年以来。通过单变量和多变量考克斯回归分析，治疗时间（因此，方案特异性治疗本身），分期和初始血清乳酸脱氢酶（LDH）的对数成为最有力的预后指标，而组织学本身与结局无显著相关性。对于1978年以来接受治疗的154例患者，按分期划分的2年EFS为97%（± 0.5%）。I期为86%（. ±.Ⅱ期为73%（. ±. 6%）的III期，和47%（±。11%）（P < .0001）。与我们以前的经验相比，我们得出结论，儿童NHL的治愈率在过去十年中增加了一倍，与现代管理。
Between 1962 and 1986, a total of 338 consecutive newly diagnosed children and adolescents with non-Hodgkin''s lymphomas (NHLs) were evaluated and treated at St Jude Children''s Research Hospital (SJCRH). Median follow-up is 6.6 years (range, 1.8 to 23 years). The patients ranged in age from 7 months to 21 years (median, 10 years), and 71% were males. All cases were staged (I to IV) by a clinical staging system. Eighteen percent were stage 1, 21% stage II, 43% stage III, and 18% stage IV. Cases frankly leukemic at diagnosis (ie., > 25% marrow blasts) were excluded from the analysis. Pathologic material from all cases reviewed and classified according to the Working Formulation. The histologic distribution of cases was as follows: 38.8% diffuse small non-cleaved cell (undifferentiated, Burkitt''s and non-Burkitt''s); 26.3% diffuse large-cell mainly immunoblastic; 28.1% lymphoblastic; and 6.8% other. Treatment policy evolved over time to a stage- and histology-specific strategy for treatment assignment, and overall results significantly improved by era from 37% (.+-. 5%) 2-year event-free survival (EFS) for patients treated before 1975 to 77% (.+-. 4%) since 1978. By univariate and multivariate Cox regression analysis, the era of treatment (hence, the protocol-specific treatment itself), the stage, and the log of the initial serum lactic dehydrogenase (LDH) emerged as the most powerful prognostic indicators, while histology per se was not significantly related to outcome. For the 154 patients treated since 1978, the 2-year EFS by stage was 97% (.+-. 3%) for stage I, 86% (.+-. 6%) for stage II, 73% (.+-. 6%) for stage III, and 47% (.+-. 11%) for stage IV (P < .0001). Compared with our previous experience, we conclude that the cure rate of childhood NHL has doubled in the last decade with modern management.