预后良好的早期 HL(无危险因素的 I 期 - II 期)
长期来讲,预后良好的早期霍奇金淋巴瘤在经过短期化疗和低剂量针对累及区域局部放疗后的疾病控制率达到85% – 90% 。[30]Meyer RM, Gospodarowicz MK, Connors JM, et al. Randomized comparison of ABVD chemotherapy with a strategy that includes radiation therapy in patients with limited-stage Hodgkin's lymphoma: National Cancer Institute of Canada Clinical Trials Group and the Eastern Cooperative Oncology Group. J Clin Oncol. 2005;23:4634-4642.http://jco.ascopubs.org/cgi/content/full/23/21/4634http://www.ncbi.nlm.nih.gov/pubmed/15837968?tool=bestpractice.com[72]Noordijk EM, Carde P, Dupouy N, et al. Combined-modality therapy for clinical stage I or II Hodgkin's lymphoma: long-term results of the European Organisation for Research and Treatment of Cancer H7 randomized controlled trials. J Clin Oncol. 2006;24:3128-3135.http://jco.ascopubs.org/cgi/content/full/24/19/3128http://www.ncbi.nlm.nih.gov/pubmed/16754934?tool=bestpractice.com[77]Engert A, Schiller P, Josting A, et al. Involved-field radiotherapy is equally effective and less toxic compared with extended-field radiotherapy after four cycles of chemotherapy in patients with early-stage unfavorable Hodgkin's lymphoma: results of the HD8 trial of the German Hodgkin's Lymphoma Study Group. J Clin Oncol. 2003;21:3601-3608.http://www.ncbi.nlm.nih.gov/pubmed/12913100?tool=bestpractice.com 复发性霍奇金淋巴瘤是导致患者治疗后15年内死亡的主要原因,被连续随访的患者很可能死于肝肿大和/或脾肿大或心脏病。[91]Ng AK, Bernardo MP, Weller E, et al. Long-term survival and competing causes of death in patients with early-stage Hodgkin's disease treated at age 50 or younger. J Clin Oncol. 2002;20:2101-2108.http://www.ncbi.nlm.nih.gov/pubmed/11956271?tool=bestpractice.com目前,临床研究正在研究治疗强度低但治愈率较高的治疗方案。
预后不良的早期霍奇金淋巴瘤(有危险因素的 I 期和 II 期霍奇金淋巴瘤)
使用单独放疗进行治疗时,大部分危险因素就已确认。采用综合疗法时,这些危险因素是否具有意义,仍未明确。
导致早期霍奇金淋巴瘤预后不良的最常见的危险因素为纵膈内有体积较大的淋巴结。安娜堡分期系统科茨沃尔德改良将纵膈内有体积较大的淋巴结定义为直径超过 T5-T6 水平直径的三分之一纵膈肿块。其他危险因素包括:高龄、B 类症状、累及淋巴结部位的数量以及病变的组织亚型。不同研究机构和研究小组对预后不良的定义是不同的。
综合疗法可提高疾病的长期控制率(80% 到 90%),即使有不良临床表现的患者也是如此。[72]Noordijk EM, Carde P, Dupouy N, et al. Combined-modality therapy for clinical stage I or II Hodgkin's lymphoma: long-term results of the European Organisation for Research and Treatment of Cancer H7 randomized controlled trials. J Clin Oncol. 2006;24:3128-3135.http://jco.ascopubs.org/cgi/content/full/24/19/3128http://www.ncbi.nlm.nih.gov/pubmed/16754934?tool=bestpractice.com[77]Engert A, Schiller P, Josting A, et al. Involved-field radiotherapy is equally effective and less toxic compared with extended-field radiotherapy after four cycles of chemotherapy in patients with early-stage unfavorable Hodgkin's lymphoma: results of the HD8 trial of the German Hodgkin's Lymphoma Study Group. J Clin Oncol. 2003;21:3601-3608.http://www.ncbi.nlm.nih.gov/pubmed/12913100?tool=bestpractice.com对于预后良好的早期霍奇金淋巴瘤,现在临床研究正在研究这种方案,以降低治疗强度,减少与治疗相关的长期并发症
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晚期霍奇金淋巴瘤(III 期和 IV 期,有或无危险因素)
晚期霍奇金淋巴瘤是一种异质性较高的疾病。总体来讲,单独化疗或采用综合疗法后,疾病的长期控制率大约为 60% 到 80%。[92]Diehl V, Franklin J, Pfreundschuh M, et al. Standard and increased-dose BEACOPP chemotherapy compared with COPP-ABVD for advanced Hodgkin's disease. N Engl J Med. 2003;348:2386-2395 (erratum in: N Engl J Med. 2005;353:744 dosage error in article text).http://content.nejm.org/cgi/content/full/348/24/2386http://www.ncbi.nlm.nih.gov/pubmed/12802024?tool=bestpractice.com[93]Canellos GP, Anderson JR, Propert KJ, et al. Chemotherapy of advanced Hodgkin's disease with MOPP, ABVD, or MOPP alternating with ABVD. N Engl J Med. 1992;327:1478-1484.http://www.ncbi.nlm.nih.gov/pubmed/1383821?tool=bestpractice.com[94]Gobbi PG, Levis A, Chisesi T, et al. ABVD versus modified Stanford V versus MOPPEBVCAD with optional and limited radiotherapy in intermediate- and advanced-stage Hodgkin's lymphoma: final results of a multicenter randomized trial by the Intergruppo Italiano Linfomi. J Clin Oncol. 2005;23:9198-9207.http://jco.ascopubs.org/cgi/content/full/23/36/9198http://www.ncbi.nlm.nih.gov/pubmed/16172458?tool=bestpractice.com现在有一个预后评价系统,包含具有独立预后价值的 7 个危险因素(低白蛋白、低血红蛋白、白细胞增多、淋巴细胞减少、男性、老年以及 IV 期疾病),这些不利因素可用于预测疾病是否会进展。[95]Hasenclever D, Diehl V. A prognostic score for advanced Hodgkin's disease. International Prognostic Factors Project on Advanced Hodgkin's Disease. N Engl J Med. 1998;339:1506-1514.http://content.nejm.org/cgi/content/full/339/21/1506http://www.ncbi.nlm.nih.gov/pubmed/9819449?tool=bestpractice.com在没有危险因素的条件下,5年无进展生存达 84%,而在有 5 个以上危险因素的条件下,5年无进展生存为 42% 。目前,临床研究根据危险因素,正在制定调整治疗方案。