NCCN T细胞淋巴瘤临床实践指南2017.2版（2）
目录
蕈样肉芽肿/Sezary综合征（MFSS）
MFSS-1
诊断
a.转化或者滤泡趋向区域的出现可能对治疗方案的选择及预后产生重要的影响，同时也应在病理报告中予以说明。
b.临床或组织学未诊断病例。Pimpinelli N, Olsen EA, Santucci M, et
al,for the International Society for Cutaneous Lymphoma.Defining early mycosis fungoides.J Am Acad Dermatol 2005;53:1053-1063.
c.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》）
d.典型的免疫表型：CD2+、CD3+、CD5+、CD7-、CD4+、CD8-(CD8+罕见)、CD30-/+、细胞毒性颗粒蛋白阴性。
e.TCR基因重排的结果应谨慎解释。TCR克隆性重排也可见于非恶性疾患，也可能不会表现于所有的蕈样肉芽肿/Sezary综合征病例。对于某些病例，检查皮肤、血液和/或淋巴结中的相同克隆可能会有帮助。
f.见HTLV-1流行地理区域地图。
MFSS-2
检查和分期
g.Sezary综合征（B2期）定义见MFSS-3。
h.见关于在T1病变中适用Sezary流失细胞术的讨论。i对于孕妇而言，许多作用于皮肤的治疗或全身治疗为禁用或其安全性不明。
应参考具体用药说明。
MFSS-3
TNMB分类及分期
j.改编自Olsen E, Vonderheid E, Pimpinelli N, et al.Blood 2007;110:1713-1722及Olsen E, Whittaker S, Kim Y, et al. J Clin Oncol 2011;29:2598-2607。
k.Sezary综合征（B2）的定义为：血液中TCR基因克隆性重排（克隆应与皮肤中的克隆相关）
l.斑片：无显著凸起或硬化的任何大小的皮肤病灶。应注意有无色素减退或色素沉着、鳞屑、结痂和/或皮肤异色。
m.斑块：任何大小的皮肤凸起或硬化病灶。应注意是否有鳞屑、结痂和/或皮肤异色。着重明确组织学特征如亲毛囊性或大细胞转化型（大细胞≥25%），CD30+或CD30-，以及皮肤溃疡等临床特征。
n.肿瘤：至少有1个直径＞1cm的实体或结节样皮损，隆起或向深部浸润生长。记录皮损的数量、皮损的总体积、最大的皮损大小、以及身体受累的区域。如果有大细胞转化的组织学证据也应当记录。鼓励做CD30表型检查。
MFSS-4
MF和SS临床分期
j.Olsen E, Vonderheid E, Pimpinelli N, et al. Blood 2007;110:1713-1722
MFSS-5
IA期的临床路径
o.在极少数确诊为单侧病变的MF，RT病例中，放疗可长期缓解症状。
p.首选在专科治疗中心接受治疗。
q.对于亲毛囊型或大细胞转化型MF,皮肤病变对局部治疗的反应性可能较差。
r.与其他NHL亚型不同，MF/SS缓解标准未被证实与其预后相关。通常情况下，根据临床状况决定继续或转换治疗方法。然而，一份详细的缓解标准提案已被公布（Olsen E,Whittaker S, Kim YH, et al.J Clin Oncol 2011;29:2598-2607）。
s.达到缓解和/或有临床获益的患者应当考虑减量或维持治疗以获得最佳缓解持续时间。通常原方案对复发的患者仍然有效。部分缓解的患者在进入难治性疾病治疗之前应当选择主要治疗列表中的其他方案以求提高疗效。初始主要治疗后疾病复发或持续的患者推荐参加临床试验。
MFSS-6
IB-IIA期的临床路径
p.首选在专科治疗中心接受治疗
r.与其他NHL亚型不同，MF/SS缓解标准未被证实与其预后相关。通常情况下，根据临床状况决定继续或转换治疗方法。然而，一份详细的缓解标准提案已被公布（Olsen E,Whittaker S, Kim YH, et al.J Clin Oncol 2011;29:2598-2607)
s.达到缓解和/或有临床获益的患者应当考虑减量或维持治疗以获得最佳缓解持续时间。通常原方案对复发的患者仍然有效。部分缓解的患者在进入难治性疾病治疗之前应当选择主要治疗列表中的其他方案以求提高疗效。初始主要治疗后疾病复发或持续的患者推荐参加临床试验。
t.当检查中怀疑临床真皮外病变时，应行影像学检查。
u.泛发性皮肤病变治疗有局部耐药的患者，耐药部位可能需要额外增加局部治疗。
v.组织学证实的亲毛囊型或大细胞转化型MF，有疾病进展的高风险。
MFSS-7
IIB期的临床路径
p.首选在专科治疗中心接受治疗。
r.与其他NHL亚型不同，MF/SS缓解标准未被证实与其预后相关。通常情况下，根据临床状况决定继续或转换治疗方法。然而，一份详细的缓解标准提案已被公布（Olsen E,Whittaker S, Kim YH, et al.J Clin Oncol 2011;29:2598-2607)
s.达到缓解和/或有临床获益的患者应当考虑减量或维持治疗以获得最佳缓解持续时间。通常原方案对复发的患者仍然有效。部分缓解的患者在进入难治性疾病治疗之前应当选择主要治疗列表中的其他方案以求提高疗效。初始主要治疗后疾病复发或持续的患者推荐参加临床试验。
t.当检查中怀疑临床真皮外病变时，应行影像学检查。
w.如果怀疑大细胞转化，则再次活检。
x.经常出现大细胞转化的组织学证据，但并不总是对应于更具侵袭性的生长速度。如果没有更具侵袭性的生长的证据，则选择SYST-CAT A或SYST-CAT B方案进行全身治疗是合适的。如果发现更具侵袭性的生长，那么在SYST-CAT C中列出的药物是首选。
y.惰性/斑块性亲毛囊性蕈样霉菌病患者（无LCT证据）在采用SYST-CAT B或SYST-CAT C之前，应首先考虑SYST-CAT A治疗。
z.肿瘤病变首选放疗。
aa.为延长缓解持续时间，可考虑在全身皮肤电子线照射（TSEBT）后辅助全身生物治疗（SYST-CAT A）。
bb.大多数患者在接受多药联合化疗之前已接受过多种治疗（SYST-CAT A/B）或联合治疗。
cc.异基因造血干细胞移植（HSCT）的作用是有争议的。进一步的信息见“讨论”章节。
MFSS-8
III期的临床路径
p.首选在专科治疗中心接受治疗。
r.与其他NHL亚型不同，MF/SS缓解标准未被证实与其预后相关。通常情况下，根据临床状况决定继续或转换治疗方法。然而，一份详细的缓解标准提案已被公布。（Olsen E,Whittaker S, Kim YH, et al.J Clin Oncol 2011;29:2598-2607)
s.达到缓解和/或有临床获益的患者应当考虑减量或维持治疗以获得最佳缓解持续时间。通常原方案对复发的患者仍然有效。部分缓解的患者在进入难治性治疗之前应当选择主要治疗列表中的其他方案以求提高疗效。初始主要治疗后疾病复发或持续的患者推荐参加临床试验。
t.当检查中怀疑临床真皮外病变时，应行影像学检查。
w.如果怀疑大细胞转化，则再次活检。
dd.异基因造血干细胞移植（HSCT）的作用是有争议的。进一步的信息见“讨论”章节。
ee.III期患者可能不能耐受作用于泛发性皮肤病变的治疗TSEBT,应用时应谨慎。在某些情况下，可以低剂量、慢分馏的应用TSEBT。
ff.中强度局部使用类固醇（± 闭合给药模式）联合使用任何一种初始治疗方法来减轻皮肤症状。红皮病样患者继发皮肤病原菌感染的风险性增高，应当考虑全身抗生素治疗。
gg.根据治疗的可获得性和症状的严重性，可在更早期（主要治疗）考虑联合治疗。
hh.低剂量阿仑单抗皮下注射引起感染等并发症的几率较低。
MFSS-9
IV期的临床路径
p.首选在专科治疗中心接受治疗。
r.与其他NHL亚型不同，MF/SS缓解标准未被证实与其预后相关。通常情况下，根据临床状况决定继续或转换治疗方法。然而，一份详细的缓解标准提案已被公布（Olsen E,Whittaker S, Kim YH, et al.J Clin Oncol 2011;29:2598-2607）。
s.达到缓解和/或有临床获益的患者应当考虑减量或维持治疗以获得最佳缓解持续时间。通常原方案对复发的患者仍然有效。部分缓解的患者在进入难治性疾病治疗之前应当选择主要治疗列表中的其他方案以求提高疗效。初始主要治疗后疾病复发或持续的患者推荐参加临床试验。
dd.异基因造血干细胞移植（HSCT）的作用是有争议的。进一步的信息见“讨论”章节。
hh.低剂量阿仑单抗皮下注射引起感染等并发症的几率较低。
ii.IV期的非Sezary/内脏疾病患者可能会出现更具侵袭性的生长特性。如果没有更具侵袭性的生长的证据，则选择SYST-CAT B方案全身治疗是合适的。如果发现侵袭性的生长，那么在SYST-CAT C中列出的药物是首选。
jj.考虑化疗后辅助全身生物治疗（SYST-CAT A）以提高缓解持续时间。
kk.若出现淋巴结和/或内脏病变或怀疑病情进展，则应根据基于疾病分布的临床需要行影像学检查。
MFSS-A（4-1）
推荐治疗方案
a.见方案的参考文献。（MFSS-A 2/4、MFSS-A 3/4和MFSS-A 4/4）
b.长期使用局部类固醇激素可能伴随皮肤萎缩和/或紫纹。这种风险随着类固醇剂量的增加而加大。在大面积皮肤表面使用大剂量类固醇可能引起全身性吸收。
c.UV的累积剂量使UV相关的皮肤恶性肿瘤发生的风险增高；因此，光疗可能不适合有泛发性鳞状上皮增生性皮肤肿瘤、基底细胞癌以及黑色素瘤病史的患者。
d.TSEBT后采用干扰素或贝沙罗汀全身治疗以维持缓解是一种通行的做法。
e.全身皮肤电子线照射（TSEBT）联合全身类视黄醇或HDAC抑制剂，如伏立诺他或罗米地辛，以及光疗联合伏立诺他或罗米地辛的安全性还不清楚。
f.光分离置换可能更适合做为血液受累（B1或B2）患者的全身治疗。
g.二期初步试验的数据显示在蕈样肉/Sezary综合征患者中，某些病人的病情出现恶化（特别是红皮病型皮肤/Sezary的病人），应该同其病情的进展相区别。Khodadoust M, Rook A, Porcu P, et al. Pembrolizumab for treatment of relapsed/refractory mycosis fungoides and Sezary syndrome:Clinical efficacy in a CITN multicenter phase 2 study [abstract].Blood 2016;125:Abstract 181.
h.大细胞转化（LCT）MF和IV期非Sezary/内脏疾病患者可能会表现更具侵袭性的长特性。一般在这种情况下，在SYST-CAT C中列出的药物是首选。
i.联合治疗方案通常只用于复发/难治性患者或皮肤外病变患者。
MFSS-A(4-2,3,4)
参考文献
作用于皮肤的治疗：
局部皮质类固醇类药物
Zackheim HS, Kashani Sabet M, Amin S. Topical corticosteroids for mycosis fungoides. Experience in 79 patients. Arch Dermatol 1998;134(8):949-954.
Zackheim HS. Treatment of patch stage mycosis fungoides with topical corticosteroids. Dermatol Ther 2003;16:283-287.
氮芥（二氯甲基二乙胺盐酸盐）
Kim YH, Martinez G, Varghese A, Hoppe RT. Topical nitrogen mustard in the management of mycosis fungoides: Update of the Stanford experience. Arch Dermatol 2003;139:165-173.
Lessin SR, Duvic M, Guitart J, et al. Topical chemotherapy in cutaneous T-cell lymphoma: positive results of a randomized, controlled, multicenter trial testing the efficacy and safety of a novel mechlorethamine, 0.02%, gel in mycosis fungoides. JAMA Dermatol 2013;149:25-32.
局部放疗
Wilson LD, Kacinski BM, Jones GW. Local superficial radiotherapy in the management of minimal stage IA cutaneous T-cell lymphoma (Mycosis Fungoides). Int J Radiat Oncol Biol Phys 1998;40:109-115.
Neelis KJ, Schimmel EC, Vermeer MH, et al. Low-dose palliative radiotherapy for cutaneous B- and T-cell lymphomas. Int J Radiat Oncol Biol Phys 2009;74:154-158.
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局部贝沙罗汀治疗
Breneman D, Duvic M, Kuzel T, et al. Phase 1 and 2 trial of bexarotene gel for skin directed treatment of patients with cutaneous T cell lymphoma. Arch Dermatol 2002;138:325-332.
Heald P, Mehlmauer M, Martin AG, et al. Topical bexarotene therapy for patients with refractory or persistent early stage cutaneous T cell lymphoma: results of the phase III clinical trial. J Am Acad Dermatol 2003;49:801-815.
他扎罗汀凝胶
Apisarnthanarax N, Talpur R, Ward S, Ni X, Kim HW, Duvic M. Tazarotene 0.1% gel for refractory mycosis fungoides lesions: an open-label pilot study. J Am Acad Dermatol 2004;50:600-607.
光疗（UVB及PUVA）
Gathers RC, Scherschun L, Malick F, Fivenson DP, Lim HW. Narrowband UVB phototherapy for early stage mycosis fungoides. J Am Acad Dermatol 2002;47:191-197.
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全身皮肤电子线照射（TSEBT）
Chinn DM, Chow S, Kim YH, Hoppe RT. Total skin electron beam therapy with or without adjuvant topical nitrogen mustard or nitrogen mustard alone as initial treatment of T2 and T3 mycosis fungoides. Int J Radiat Oncol Biol Phys 1999;43:951-958.
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全身治疗
阿仑单抗（适用于Sezary综合征±淋巴结病变）
Lundin J, Hagberg H, Repp R, et al. Phase 2 study of alemtuzumab (anti-CD52 monoclonal antibody) in patients with advanced mycosis fungoides/Sezary syndrome. Blood 2003;101:4267-4272.
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硼替佐米
Zinzani PL, Musuraca G, Tani M, et al. Phase II trial of proteasome inhibitor bortezomib in patients with relapsed or refractory cutaneous T-cell lymphoma. J Clin Oncol 2007;25:4293-4297.
Brentuximab vedotin
Duvic M, Tetzlaff M, Gangar P, et al. Results of a Phase II Trial of Brentuximab Vedotin for CD30+ Cutaneous T-Cell Lymphoma and Lymphomatoid Papulosis. J Clin Oncol 2015;33:3759-3765
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类视黄醇
Zhang C, Duvic M. Treatment of cutaneous T-cell lymphoma with retinoids. Dermatol Ther 2006;19:264-271.
Duvic M, Martin AG, Kim Y, et al. Phase 2 and 3 clinical trial of oral bexarotene (Targretin capsules) for the treatment of refractory or persistent early-stage cutaneous T-cell lymphoma. Arch Dermatol 2001;137:581-593. Duvic M, Hymes K, Heald P, et al. Bexarotene is effective and safe for treatment of refractory advancedstage cutaneous T-cell lymphoma: multinational phase II-III trial results. J Clin Oncol 2001;19:2456-2471.
干扰素
Olsen EA. Interferon in the treatment of cutaneous T-cell lymphoma. Dermatol Ther 2003;16:311-321. Kaplan EH, Rosen ST, Norris DB, et al. Phase II study of recombinant human interferon gamma for treatment of cutaneous T-cell lymphoma. J Natl Cancer Inst 1990;82:208-212
伏立诺他
Duvic M, Talpur R, Ni X, et al. Phase 2 trial of oral vorinostat (suberoylanilide hydroxamic acid, SAHA) for refractory cutaneous T-cell lymphoma (CTCL). Blood 2007;109:31-39.
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Duvic M, Olsen EA, Breneman D, et al. Evaluation of the long-term tolerability and clinical beneft of vorinostat in patients with advanced cutaneous T-cell lymphoma. Clin Lymphoma Myeloma 2009;9:412-416.
罗米地辛
Piekarz RL, Frye R, Turner M, et al. Phase II Multi-Institutional Trial of the Histone Deacetylase Inhibitor Romidepsin As Monotherapy for Patients With Cutaneous T-Cell Lymphoma. J Clin Oncol 2009;27:5410-5417.
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体外光分离置换法（ECP）
Edelson R, Berger C, Gasparro F, et al. Treatment of cutaneous T-cell lymphoma by extracorporeal photochemotherapy. Preliminary results. N Engl J Med 1987;316:297-303.
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甲氨蝶呤
Zackheim HS, Kashani-Sabet M, Hwang ST. Low-dose methotrexate to treat erythrodermic cutaneous T-cell lymphoma: results in twenty-nine patients. J Am Acad Dermatol 1996;34:626-631. Zackheim HS, Kashani-Sabet M, McMillan A. Low-dose methotrexate to treat mycosis fungoides: a retrospective study in 69 patients. J Am Acad Dermatol 2003;49:873-878
脂质体阿霉素
Wollina U, Dummer R, Brockmeyer NH, et al. Multicenter study of pegylated liposomal doxorubicin in patients with cutaneous T-cell lymphoma. Cancer 2003;98:993-1001.
Quereux G, Marques S, Nguyen J-M, et al. Prospective multicenter study of pegylated liposomal doxorubicin treatment in patients with advanced or refractory mycosis fungoides or Sezary syndrome. Arch Dermatol 2008;144:727-733.
Dummer R, Quaglino P, Becker JC, et al. Prospective international multicenter phase II trial of intravenous pegylated liposomal doxorubicin monochemotherapy in patients with stage IIB, IVA, or IVB advanced mycosis fungoides: fnal results from EORTC 21012. J Clin Oncol 2012;30:4091-4097.
吉西他滨
Duvic M, Talpur R, Wen S, Kurzrock R, David CL, Apisarnthanarax N. Phase II evaluation of gemcitabine monotherapy for cutaneous T-cell lymphoma. Clin Lymphoma Myeloma 2006;7:51-58.
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Awar O, Duvic M. Treatment of transformed mycosis fungoides with intermittent low-dose gemcitabine. Oncology 2007;73:130-135.
喷司他丁
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硼替佐米
Zinzani PL, Musuraca G, Tani M, et al. Phase II trial of proteasome inhibitor bortezomib in patients with relapsed or refractory cutaneous T-cell lymphoma. J Clin Oncol 2007;25:4293-4297.
替莫唑胺
Tani M, Fina M, Alinari L, Stefoni V, Baccarani M, Zinzani PL. Phase II trial of temozolomide in patients with pretreated cutaneous T-cell lymphoma. Haematologica 2005;90(9):1283-1284.
Querfeld C, Rosen ST, Guitart J, et al. Multicenter phase II trial of temozolomide in mycosis fungoides/ sezary syndrome: correlation with O6-methylguanine-DNA methyltransferase and mismatch repair proteins. Clin Cancer Res 2011;17:5748-5754.
普拉曲沙
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派姆单抗
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联合治疗
作用于皮肤的治疗+全身治疗
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全身治疗+全身治疗
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异基因干细胞移植
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MSAA-B
MF/SS支持治疗
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