Weitzman Sheila, Suryanarayan Kaveri, Weinstein Howard J
Division of Hematology/Oncology, The Hospital For Sick Children, 555 University Avenue, Toronto, ON M5G 1X8, Canada.
Curr Oncol Rep. 2002 Mar;4(2):107-13. doi: 10.1007/s11912-002-0071-6.
The use of effective combination chemotherapy for all stages and subtypes of non-Hodgkin"s lymphoma (NHL) in children has resulted in a striking improvement in cure rates. Event-free survival now ranges from 70% to 90%, depending on the stage of disease and the NHL subtype. Risk-adapted therapy has resulted in a dramatic improvement in outcome for high-risk patients, at the cost of significantly increased short-term toxicity, and a reduction of therapy and toxicity for the lower-risk patient, while maintaining the excellent cure rate. Successful risk allocation of patients is dependent on the identification and continual validation of prognostic factors. The specific treatment protocol is the single most important factor predicting outcome today. Traditional prognostic factors such as stage and tumor burden are useful in selecting the intensity and length of therapy, rather than as a major indicator of likelihood of survival. In order to further improve cure rates and decrease toxicity, new biologic prognosticators need to be found and validated. Some promising avenues for study appear to be the presence or absence of adhesion molecules and of aberrant proteins that are specific to subtypes of lymphomas, such as soluble CD30 and anaplastic lymphoma kinase (ALK), the molecular classification of lymphomas on the basis of gene expression, and the evaluation of biologic markers for measuring early response to therapy.
对儿童非霍奇金淋巴瘤（NHL）的所有分期和亚型采用有效的联合化疗，已使治愈率显著提高。无事件生存率目前在70%至90%之间，具体取决于疾病分期和NHL亚型。风险适应性治疗已使高危患者的治疗结果有了显著改善，但代价是短期毒性显著增加，同时降低了低危患者的治疗强度和毒性，且保持了极高的治愈率。成功地对患者进行风险分类取决于对预后因素的识别和持续验证。具体的治疗方案是当今预测治疗结果的最重要单一因素。传统的预后因素，如分期和肿瘤负荷，在选择治疗强度和疗程方面有用，但并非生存可能性的主要指标。为了进一步提高治愈率并降低毒性，需要找到并验证新的生物学预后指标。一些有前景的研究途径似乎包括淋巴瘤亚型特异性的黏附分子和异常蛋白的有无，如可溶性CD30和间变性淋巴瘤激酶（ALK），基于基因表达的淋巴瘤分子分类，以及用于评估治疗早期反应的生物学标志物。
