预后通常与淋巴瘤的类型、疾病分期、治疗及合并症有关。
预后较差见于以下情况:
B症状(体重减轻,盗汗及发热)
淋巴结肿大
器官肿大症
皮肤变化
东部肿瘤协作组(ECOG)体能状态评分低
有相当大比例的侵袭性淋巴瘤患者通过现代的化疗可达到治愈。 惰性淋巴瘤通常是不可治愈的。 针对侵袭性淋巴瘤提出了国际预后指标(IPI)[147]The International Non-Hodgkin's Lymphoma Prognostic Factors Project. A predictive model for aggressive non-Hodgkin's lymphoma. N Engl J Med. 1993 Sep 30;329(14):987-94.http://www.nejm.org/doi/full/10.1056/NEJM199309303291402#t=articlehttp://www.ncbi.nlm.nih.gov/pubmed/8141877?tool=bestpractice.com 及年龄矫正后的预后指标,提供了判断预后的标准。 对于滤泡性淋巴瘤,也有滤泡性淋巴瘤国际预后指标(FLIPI)可用于判断预后。[148]Solal-Celigny P, Roy P, Colombat P, et al. Follicular lymphoma international prognostic index. Blood. 2004 Sep 1;104(5):1258-65.http://bloodjournal.hematologylibrary.org/cgi/content/full/104/5/1258http://www.ncbi.nlm.nih.gov/pubmed/15126323?tool=bestpractice.com
IPI模型包括反应肿瘤生长及潜在侵袭性的临床特征(肿瘤分期,血清乳酸脱氢酶[LDH]水平,以及结外受累病变的数量),肿瘤对患者的影响(患者体能状态)以及患者耐受强化化疗的能力(年龄及体能状态)。[147]The International Non-Hodgkin's Lymphoma Prognostic Factors Project. A predictive model for aggressive non-Hodgkin's lymphoma. N Engl J Med. 1993 Sep 30;329(14):987-94.http://www.nejm.org/doi/full/10.1056/NEJM199309303291402#t=articlehttp://www.ncbi.nlm.nih.gov/pubmed/8141877?tool=bestpractice.com
FLIPI 模型包括 5 个不良预后因素:年龄(大于 60 岁),Ann Arbor 分期(III 期或 IV 期),血红蛋白水平(小于 120 g/L),受累淋巴结区数量(大于 4 个)及血清 LDH 水平(高于正常值)。 分为低危(0-1个危险因素)、中危(2个危险因素)及高危(3个或更多危险因素)三类。[148]Solal-Celigny P, Roy P, Colombat P, et al. Follicular lymphoma international prognostic index. Blood. 2004 Sep 1;104(5):1258-65.http://bloodjournal.hematologylibrary.org/cgi/content/full/104/5/1258http://www.ncbi.nlm.nih.gov/pubmed/15126323?tool=bestpractice.com
ECOG体能状态评分是侵袭性淋巴瘤判断预后的重要参数。 IPI能够很好的判断预后,因此可能会影响治疗策略;例如,对于体能状态好的患者可能会选择更积极的治疗和/或骨髓移植,而对体能状态差的患者可能会采取姑息治疗。 此外,许多临床研究方案排除了ECOG评分在3分及以上患者。
特殊亚型
弥漫性大B细胞淋巴瘤(DLBCL)
I 至 II 期非肿块型(直径<10 cm),接受 R-CHOP-21(利妥昔单抗+环磷酰胺、多柔比星、长春新碱、泼尼松龙给药 21 天)连续 6 至 8 个疗程或 R-CHOP-21 连续 3 个疗程加上受累野放疗 (30-40 Gy) 治疗,治愈率为 70-85%。[149]Wu HJ, Zhang QY, Chen DF et al. Comparison of rituximab plus CHOP regimen and CHOP regimen alone for treatment of newly diagnosed patients with diffuse large B-cell lymphoma [in Chinese]. Ai Zheng. 2005 Dec;24(12):1498-502.http://www.ncbi.nlm.nih.gov/pubmed/16351800?tool=bestpractice.com
III-IV期应用R-CHOP-21方案6-8疗程,治愈率接近40%[51]Sehn LH, Donaldson J, Chanabhai M, et al. Introduction of combined CHOP plus rituximab therapy dramatically improved outcome of diffuse large B-cell lymphoma in British Columbia. J Clin Oncol. 2005 Aug 1;23(22):5027-33.http://www.ncbi.nlm.nih.gov/pubmed/15955905?tool=bestpractice.com
若未进行中枢神经系统(CNS)预防性治疗,多达 27%的高危患者会出现CNS 复发。[52]Arkenau HT, Chong G, Cunningham D, et al. The role of intrathecal chemotherapy prophylaxis in patients with diffuse large B-cell lymphoma. Ann Oncol. 2007 Mar;18(3):541-5.http://www.ncbi.nlm.nih.gov/pubmed/17164228?tool=bestpractice.com
R-ICE(利妥昔单抗、异环磷酰胺、卡铂和依托泊苷),用于治疗 R-CHOP-21 治疗后复发的病例,治愈率为 20-50%。[54]Kewalramani T, Zelenetz AD, Nimer SD, et al. Rituximab and ICE as second-line therapy prior to autologous stem cell transplantation for relapsed or primary refractory diffuse large B-cell lymphoma. Blood. 2004 May 15;103(10):3684-8.http://www.bloodjournal.org/content/103/10/3684.longhttp://www.ncbi.nlm.nih.gov/pubmed/14739217?tool=bestpractice.com
原发纵隔LBCL较DLBCL预后好。[150]Savage KJ, Al-Rajhi N, Voss N, et al. Favorable outcome of primary mediastinal large B-cell lymphoma in a single institution: the British Columbia experience. Ann Oncol. 2006 Jan;17(1):123-30.http://www.ncbi.nlm.nih.gov/pubmed/16236753?tool=bestpractice.com
原发中枢神经系统型非霍奇金淋巴瘤
最有效的治疗方案是甲氨蝶呤为基础的化疗联合全脑放疗,2年生存率为40%-70%。[151]Ferreri AJ, Abrey LE, Blay J-Y, et al. Management of primary central nervous system lymphoma: a summary statement from the 8th International Conference on Malignant Lymphoma. J Clin Oncol. 2003 Jun 15;21(12):2407-14.http://www.ncbi.nlm.nih.gov/pubmed/12805341?tool=bestpractice.com
原发渗出性淋巴瘤/体腔淋巴瘤
中位生存期仅6个月。[152]Simonelli C, Spina M, Cinelli R, et al. Clinical features and outcome of primary effusion lymphoma in HIV-infected patients: a single-institution study. J Clin Oncol. 2003 Nov 1;21(21):3948-54.http://www.ncbi.nlm.nih.gov/pubmed/14581418?tool=bestpractice.com
伯基特淋巴瘤及伯基特样淋巴瘤
套细胞淋巴瘤
皮下脂腺炎样外周T细胞淋巴瘤
系统性间变性大T细胞淋巴瘤
肠病型T细胞淋巴瘤/肠道T细胞淋巴瘤
预后差,平均生存期小于 1 年。[153]Gale J, Simmonds PD, Mead GM, et al. Enteropathy-type intestinal T-cell lymphoma: clinical features and treatment of 31 patients in a single center. J Clin Oncol. 2000 Feb;18(4):795-803.http://www.ncbi.nlm.nih.gov/pubmed/10673521?tool=bestpractice.com
蕈样肉芽肿/Sézary 综合征
研究发现,IV 期疾病、年龄大于 60 岁、转化为大细胞淋巴瘤以及 LDH 升高是生存期降低的独立预后标志。 III 期疾病患者的生存情况稍好于 IIB 期疾病患者。[154]Scarisbrick JJ, Prince HM, Vermeer MH, et al. Cutaneous Lymphoma International Consortium study of outcome in advanced stages of mycosis fungoides and Sézary syndrome: effect of specific prognostic markers on survival and development of a prognostic model. J Clin Oncol. 2015 Nov 10;33(32):3766-73.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4979132/http://www.ncbi.nlm.nih.gov/pubmed/26438120?tool=bestpractice.com[155]Wilcox RA. Cutaneous T-cell lymphoma: 2016 update on diagnosis, risk-stratification, and management. Am J Hematol. 2016 Jan;91(1):151-65.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4715621/http://www.ncbi.nlm.nih.gov/pubmed/26607183?tool=bestpractice.com
滤泡性淋巴瘤
新的治疗手段可延长生存,甚至在部分患者中可提高治愈率。[156]Liu Qi, Fayad L, Cabanillas F, et al. Improvement of overall and failure-free survival in stage IV follicular lymphoma: 25 years of treatment experience at The University of Texas M.D. Anderson Cancer Center. J Clin Oncol. 2006 Apr 1;24(10):1582-9.http://www.ncbi.nlm.nih.gov/pubmed/16575009?tool=bestpractice.com