NCCN T细胞淋巴瘤临床实践指南2017.2版（1）
目录
外周T细胞淋巴瘤（TCEL）
TCEL-1
诊断和亚型
注解：
a.见免疫表型/基因检测在成熟B细胞与NK/T细胞肿瘤的鉴别诊断中的应用。（见《NCCN B细胞淋巴瘤指南》）
b.T细胞受体基因重排本身不足以构成诊断，因为这常见于再激活/炎症过程中。
c.见HTLV-1流行地理区域地图。
d.主要侵犯皮肤的外周T细胞淋巴瘤非常异质，遵循这个指南并不是最佳的治疗手段。
e.AITL偶尔合并存在DLBCL。应进行EBV检测及适当的免疫组化检查。
f.MEITL仅最近被分离，其本身实体和最佳治疗手段尚未定论。
TCEL-2
检查
g.鞘内预防性注射在PTCL中的作用尚不清楚。
h.见“国际预后指数”（TCEL-A）。
TCEL-3
分期与诱导治疗
f.MEITL仅最近被分离，其本身实体和最佳治疗手段尚未定论。i.ALCL-ALK阴性伴DUSP22重排的患者预后与ALK阳性的预后更为类似，可依据ALCL-ALK阳性的方法予以治疗（Parrilla Castellar ER, Jaffe ES, Said JW, et al.ALK-negative anaplastic large cell lymphoma is a genetically heterogeneous disease with widely disparate clinical outcomes. Blood 2014;124:1473-1480.）
j.对某些患者（老年患者或有合并症）考虑进行有关皮质类固醇单药的临床试验以缓解症状。
k.见建议治疗方案（TCEL-B）
l.见非霍奇金淋巴瘤卢加诺响应标准（LYMP-B）
m.局部区域在大剂量化疗前后可行放疗。
TCEL-4
复发/难治性疾病的附加治疗
l.见非霍奇金淋巴瘤卢加诺响应标准（LYMP-B）
m.局部区域在大剂量化疗前后可行放疗。
TCEL-A
预后指数
a.The International Non-Hodgkin's Lymphoma Prognostic Factors Project.A predictive model for aggressive non-hodgkin's lymphoma.N Engl J Med1993;329:987-994.
b.Gallamini A, Stelitano C, Calvi R, et al.Peripheral T-cell lymphoma unspecified (PTCL-U):A new prognostic model from a retrospective multicentric clinical study.Blood 2004;103:2474-2479.EL-A）。
TCEL-B（5-1）
推荐治疗方案
a.见方案的参考文献（TCEL-B 5/5）
b.尽管ALCL，ALK+采用CHOP-21和CHOEP-21方案有良好预后，但其他PTCL组织学类型采用这些方案对并不能达到相同的良好预后；因此，这些其他组织学类型的治疗首选临床试验。
c.ALCL-ALK阴性伴DUSP22重排的患者预后与ALK阳性的预后更为类似，可依据ALCL-ALK阳性的方法予以治疗（Parrilla Castellar ER, Jaffe ES, Said JW, et al.ALK-negative anaplastic large cell lymphoma is a genetically heterogeneous disease with widely disparate clinical outcomes. Blood 2014;124:1473-1480.）
d.MEITL仅最近被分离，其本身实体和最佳治疗手段尚未定论。e.CHOP继以LIVE方案包括HSCT。
TCEL-B（5-2）
PTCL-非特指性，EATL，MEITL的推荐治疗方案
a.见方案的参考文献（TCEL-B 5/5）
d.MEITL仅最近被分离，其本身实体和最佳治疗手段尚未定论。
f.数据显示当GVD（吉西他滨、地塞米松、顺铂）与非共轭的抗CD30单克隆抗体结合用于霍奇金淋巴瘤的治疗时可能有较明显肺毒性（(Blum KA, Jung SH, Johnson JL, et al. Ann Oncol 2010;21:2246-2254)）一个包含吉西他滨和阿霉素脂质体的类似的治疗方案也许可用于T细胞淋巴瘤，但建议在开始治疗方案前3-4周先用Brentuximab vedotin。
g.疗效已在小型临床试验中得到证实，但尚需进一步大型临床试验证实。
TCEL-B(5-3)
AITL的推荐治疗方案
a.见方案的参考文献（TCEL-B 5/5）
g.疗效已在小型临床试验中得到证实，但尚需进一步大型临床试验证实。
h.对于AITL患者，普拉曲沙的疗效有限。
i.密切随访肾功能。
TCEL-B（5-4）
ALCL的推荐治疗方案
a.见方案的参考文献（TCEL-B 5/5）
g.疗效已在小型临床试验中得到证实，但尚需进一步大型临床试验证实。
TCEL-B（5-5）
参考文献：
一线治疗方案
CHOP
Savage KJ, Chhanabhai M, Gascoyne RD, Connors JM. Characterization of peripheral T-cell lymphomas in a single North American institution by the WHO classification. Ann Oncol 2004;15:1467-1475.
CHOP或CHOP-14加或不加依托泊苷
Pfreundschuh M, Trümper L, Kloess M, Schmits R, et al. German High-Grade Non-Hodgkin's Lymphoma Study Group. Two-weekly or 3-weekly CHOP chemotherapy with or without etoposide for the treatment of young patients with good-prognosis (normal LDH) aggressive lymphomas: results of the NHL-B1 trial of the DSHNHL. Blood 2004;104:626-33.
Pfreundschuh M, Trümper L, Kloess M, Schmits R, et al. German High-Grade Non-Hodgkin's Lymphoma Study Group. Two-weekly or 3-weekly CHOP chemotherapy with or without etoposide for the treatment of elderly patients with aggressive lymphomas: Results of the NHL-B2 trial of the DSHNHL. Blood 2004;104:634-41. Schmitz N, Trumper L, Ziepert M, et al. Treatment and prognosis of mature T-cell and NK-cell lymphoma: an analysis of patients with T-cell lymphoma treated in studies of the German High-Grade Non-Hodgkin Lymphoma Study Group. Blood 2010;116:3418-3425.
CHOP方案治疗后使用IVE方案
Sieniawski M et al. Evaluation of enteropathy-associated T-cell lymphoma comparing standard therapies with a novel regimen including autologous stem cell transplantation. Blood 2010;115:3664-3670.
剂量调整的EPOCH
Dunleavy K, Shovlin M, Pittaluga S, et al. DA-EPOCH Chemotherapy is highly effective in ALK-positive and ALK-negative ALCL: Results of a prospective study of PTCL subtypes in adults [abstract]. Blood 2011;118:Abstract 1618.
Wilson WH, Bryant G, Bates S, et al. EPOCH chemotherapy: toxicity and efficacy in relapsed and refractory non-Hodgkin's lymphoma. J Clin Oncol 1993;11:1573-582.
Peng YL, Huang HQ, Lin XB, et al. [Clinical outcomes of patients with peripheral T-cell lymphoma (PTCL) treated by EPOCH regimen]. Ai Zheng 2004;23:943-946.
HyperCVAD与大剂量甲氨喋呤和阿糖胞苷交替治疗
Escalon MP, Liu NS, Yang Y, et al. Prognostic factors and treatment of patients with T-cell non-Hodgkin lymphoma: the M. D. Anderson Cancer Center experience. Cancer 2005;103:2091-2098.
Pozadzides JV, Perini G, Hess M, et al. Prognosis and treatment of patients with peripheral T-cell lymphoma: The M. D. Anderson Cancer Center experience [abstract]. J Clin Oncol 2010;28: Abstract 8051.
二线治疗方案阿仑单抗
Enblad G, Hagberg H, Erlanson M, et al. A pilot study of alemtuzumab (anti-CD52 monoclonal antibody) therapy for patients with relapsed or chemotherapy-refractory peripheral T-cell lymphomas. Blood 2004;103:2920-2924.
贝利司他
O'Connor OA, Horwitz S, Masszi T, et al. Belinostat in patients with relapsed or refractory peripheral T-cell lymphoma: Results of the pivotal phase II BELIEF (CLN-19) study. J Clin Oncol 2015;33:2492-2499.
苯达莫司汀
Damaj G, Gressin R, Bouabdallah K, et al. Results from a prospective, open-label, phase II trial of bendamustine in refractory or relapsed T-cell lymphomas: the BENTLY trial. J Clin Oncol 2013;31:104-110.
布妥昔单抗酯
Pro B, Advani R, Brice P, et al. Brentuximab vedotin (SGN-35) in patients with relapsed or refractory systemic anaplastic large-cell lymphoma: Results of a phase II study. J Clin Oncol 2012;30:2190-2196.
Jacobsen ED, Advani RH, Oki Y, et al. A Phase 2 Study of Brentuximab Vedotin in Patients with Relapsed or Refractory CD30-Positive Non-Hodgkin Lymphomas: Interim Results [abstract]. Blood 2012;120: Abstract 2746. Advani RH, Brice P, Bartlett NL, et al. Three-year survival results from an ongoing phase 2 study of brentuximab vedotin in patients with relapsed or refractory systemic anaplastic large cell lymphoma. Blood 2013;122:1809.
Horwitz SM, Advani RH, Bartlett NL, et al. Objective responses in relapsed T-cell lymphomas with single agent brentuximab vedotin. Blood 2014;123 3095-3100.
环孢素用于AITL
Advani R, Horwitz S, Zelenetz A, Horning SJ. Angioimmunoblastic T cell lymphoma: treatment experience with cyclosporine. Leuk Lymphoma 2007;48:521-525.
DHAP（地塞米松、顺铂、阿糖胞苷）
Velasquez WS, Cabanillas F, Salvador P, et al. Effective salvage therapy for lymphoma with cisplatin in combination with high-dose Ara-C and dexamethasone (DHAP). Blood 1988;71:117-122.
Mey UJ, Orlopp KS, Flieger D, et al. Dexamethasone, high-dose cytarabine, and cisplatin in combination with rituximab as salvage treatment for patients with relapsed or refractory aggressive non-Hodgkin's lymphoma. Cancer Invest 2006;24:593-600.
ESHAP（依托泊苷、甲泼尼龙、阿糖胞苷、顺铂）
Velasquez WS, McLaughlin P, Tucker S, et al. ESHAP - an effective chemotherapy regimen in refractory and relapsing lymphoma: a 4-year follow-up study. J Clin Oncol 1994;12:1169-1176.
吉西他滨
Zinzani PL, Baliva G, Magagnoli M, et al. Gemcitabine treatment in pretreated cutaneous T-cell lymphoma: Experience in 44 patients. J Clin Oncol 2000;18:2603-2606.
Zinzani PL, Magagnoli M, Bendandi M, et al. Therapy with gemcitabine in pretreated peripheral T-cell lymphoma patients. Ann Oncol 1998;9:1351-1353.
GDP（吉西他滨、地塞米松、顺铂）
Crump M, Baetz T, Couban S, et al. Gemcitabine, dexamethasone, and cisplatin in patients with recurrent or refractory aggressive histology B-cell non-Hodgkin lymphoma: a Phase II study by the National Cancer Institute of Canada Clinical Trials Group (NCIC-CTG). Cancer 2004;101:1835-1842.
Dong M, He XH, Liu P, et al. Gemcitabine-based combination regimen in patients with peripheral T-cell lymphoma. Med Oncol 2013;30:351.
Connors JM, Sehn LH, Villa D, et al. Gemcitabine,dexamethasone, and cisplatin (GDP) as secondary chemotherapy in relapsed/refractory peripheral T-cell lymphoma [abstract]. Blood 2013;122:Abstract 4345.
GVD（吉西他滨、长春瑞滨、阿霉素脂质体）
Qian Z, Song Z, Zhang H, et al. Gemcitabine, navelbine, and doxorubicin as treatment for patients with refractory or relapsed T-cell lymphoma. Biomed Res Int. 2015;2015:606752.
GemOx（吉西他滨、奥沙利铂）
Lopez A, Gutierrez A, Palacios A, et al. GEMOX-R regimen is a highly effective salvage regimen in patients with refractory/relapsing diffuse large-cell lymphoma: A phase II study. Eur J Haematol 2008;80:127-132.
ICE（异环磷酰胺、卡铂、依托泊苷）
Zelenetz AD, Hamlin P, Kewalramani T, et al. Ifosfamide, carboplatin, etoposide (ICE)-based second-line chemotherapy for the management of relapsed and refractory aggressive non-Hodgkin's lymphoma. Ann Oncol 2003;14[suppl 1]:i5-10.
普拉曲沙
O'Connor OA, Pro B, Pinter-Brown L, et al. Pralatrexate in patients with relapsed or refractory peripheral T-cell lymphoma: Results from the pivotal PROPEL study. J Clin Oncol 2011;29:1182-1189.
罗米地辛
Coiffier B, Pro B, Prince HM, et al. Results From a Pivotal, Open-Label, Phase II Study of Romidepsin in Relapsed or Refractory Peripheral T-Cell Lymphoma After Prior Systemic Therapy. J Clin Oncol 2012;30:631-636.Coiffier B, Pro B, Prince HM, et al. Romidepsin for the treatment of relapsed/refractory peripheral T-cell lymphoma: pivotal study update demonstrates durable responses]. J Hematol Oncol 2014;7:11.S
乳房植入物相关的间变性大细胞淋巴瘤（BIAA）
BIAA-1
临床表现、初始检查与病理检查
a.乳腺实质或淋巴结受累的罕见病例可能具有更符合全身性ALK阳性的ALCL的侵袭性疾病过程。针对这些病例的最佳治疗方案尚未明确，应予个体化处理。
b.大多数情况见于触诊可触及的植入物(Miranda RN, et al. Breast implant-associated anaplastic large-cell lymphoma: long-term follow-up of 60patients. J Clin Oncol 2014;32:114–120).
c.Adrada BE, et al. Breast implant-associated anaplastic large cell lymphoma: sensitivity, specificity, and findings of imaging studies in 44 patients. Breast Cancer Res Treat 2014;147:1–14.
d.Miranda RN, et al. Breast implant-associated anaplastic large-cell lymphoma: long-term follow-up of 60 patients. J Clin Oncol 2014;32:114–120
BIAA-2
淋巴瘤检查和分期、治疗、辅助治疗、随访
e.FDA推荐所有的BIA-ALCL病例均向PROFILE Registry报道www.thepsf.org/PROFILE
f.对于乳房植入物相关性间变细胞淋巴瘤，仅特定病例才需要骨髓活检。
g.例如肿瘤医师、外科肿瘤医师、整形外科医师、血液病理学家
h.大约4.6%的病例在对侧乳房发现淋巴瘤(Clemens MW, Medeiros LJ, Butler CE, et al. Complete surgical excision is essential for the management of patients with breast implant–associated anaplastic large-cell lymphoma. J Clin Oncol 2016; 34:160-168)
i.Clemens MW, Medeiros LJ, Butler CE, et al. Complete surgical excision is essential for the management of patients with breast implant–associated anaplastic large-cell lymphoma. J Clin Oncol 2016; 34:160-168
j.Pro B, Advani R, Brice P, et al. Brentuximab vedotin (SGN-35) in patients with relapsed or refractory systemic anaplastic large-cell lymphoma: results of a phase II study.J Clin Oncol 2012;30:2190-2196. Pro B, Advani R, Brice P, et al. Four-year survival data from an ongoing pivotal phase 2 study of brentuximab vedotin in patients with relapsed or refractory systemic anaplastic large cell lymphoma [abstract]. Blood 2014 124:Abstract 3095.
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