NCCN T细胞淋巴瘤临床实践指南2017.2版（3）
目录
原发性皮肤CD30阳性T细胞淋巴增殖性疾病（PCTLD）
PCTLD-1
概述&定义
a.Ralfkiaer E, Willemze R, Paulli M, Kadin ME. Primary cutaneous CD30-positive T-cell lymphoproliferative disorders. In: Swerdlow SH, Campo E, Harris NL, et al.,eds. WHO classification of tumours of haematopoietic and lymphoid tissues (ed 4th). Lyon: IARC; 2008:300-301.
b.Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 2016;127:2375-2390.
c.Benner MF, Willemze R. Applicability and prognostic value of the new TNM classification system in 135 patients with primary cutaneous anaplastic large cell lymphoma. Arch Dermatol 2009;145:1399-1404.
d.Kempf W, Pfaltz K, Vermeer MH, et al. EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. Blood 2011;118:4024-4035.
e.由于重叠的免疫表型和形态，需要注意不要诊断淋巴结中的CD30 + T细胞作为HL。(Eberle FC, Song JY, Xi L, et al. Nodal involvement by cutaneous CD30-positive T-cell lymphoma mimicking classical Hodgkin lymphoma. Amer J Surg Pathol 2012;36:716-725.)
PCTLD-2
诊断
f.见“免疫分型和基因检测在成熟B细胞和NK/T细胞淋巴瘤的鉴别诊断中的应用”（见《 B细胞淋巴瘤指南》）。
g.典型免疫表型：CD30+（>75%的细胞）、CD2/CD5/CD3的CD4+变型缺失、CD8+（<5%）细胞毒性颗粒蛋白阳性。
h.PC-ALCL和LyP中通常不存在ALK1阳性和t(2;5)易位。
i.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。
j.LyP不被认为是恶性疾病；但是，它与其他淋巴恶性肿瘤（蕈样肉芽肿或PC-ALCL）存在关联。只有存在相关全身性淋巴瘤怀疑时才进行LyP方面的分期研究。
PCTLD-3
检查
e.由于免疫表型和形态的重叠，需要谨慎地避免将淋巴结中的CD30+ T细胞诊断为HL。(Eberle FC, Song JY, Xi L, et al.Nodal involvement by cutaneous CD30-positive T-cell lymphoma mimicking classical Hodgkin lymphoma.Amer J Surg Pathol 2012;36:716-725.)
j.LyP不被认为是恶性疾病；但是，它与其他淋巴恶性肿瘤（蕈样肉芽肿或PC-ALCL）存在关联。只有存在相关全身性淋巴瘤怀疑时才进行LyP方面的分期研究。
k.病灶大小和数量的监测将有助缓解评估。
l.考虑全身性ALCL、PC-ALCL区域巴结受累或转化性MF淋巴结受累。
m.如果仅在引流淋巴结中，考虑PC-ALCL。
n.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。
o.仅用于排除相关淋巴瘤。
PCTLD-4
原发皮肤及区域淋巴结受累皮肤ALCL的临床路径
p.最高达44%的病例可出现病灶进展。
q.见“放射治疗原则”（LYMP-C）。
r.来自病例报告的有限数据（如贝沙罗汀）。
s.蕈样肉芽肿可随时间推移而出现；随访期间应持续进行全面的皮肤检查。
t.从皮肤病治疗中达到缓解和/或有临床获益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。
PCTLD-5
LyP亚型的临床路径
r.来自病例报告的有限数据（如贝沙罗汀）。
u.Kempf W, Pfaltz K, Vermeer MH, et al.EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma.Blood 2011;118:4024-4035.
v.由于二次淋巴恶性肿瘤的风险很高，需要终身随访；随访期间应持续进行全面的皮肤检查。
w.达到缓解和/或有临床获益的患者可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。
PCTLD-A
参考文献：
一般方法/治疗概述
Kempf W, Pfaltz K, Vermeer MH et al. EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30+ lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma. Blood 2011;118:4024-4035.
Vergier B, Beylot-Barry M, Pulford K, et al. Statistical evaluation of diagnostic and prognostic features of CD30+ cutaneous lymphoproliferative disorders: a clinicopathologic study of 65 cases. Am J Surg Pathol 1998;22:1192-1202.
Liu HL, Hoppe RT, Kohler S, et al. CD30+ cutaneous lymphoproliferative disorders: the Stanford experience in lymphomatoid papulosos and primary cutaneous anaplastic large cell lymphoma. J Am Acad Dermatol 2003;49:1049-1058.
Woo DK, Jones CR, Vanoli-Stolz MN, et al. Prognostic factors in primary cutaneous anaplastic large cell lymphoma: characterization of clinical subset with worse outcome. Arch Dermatol 2009;145:667-674.
作用于皮肤的治疗局部类固醇
Paul MA, Krowchuk DP, Hitchcock MG, et al. Lymphomatoid papulosis: successful weekly pulse superpotent topical corticosteroid therapy in three pediatric patients. Pediatr Dermatol 1996;13:501-506.
光疗
Wantzin GL, Thomsen K. PUVA-treatment in lymphomatoid papulosis. Br J Dermatol 1982;107:687-690.
局部氮芥治疗
Vonderheid EC, Tan ET, Kantor AF, et al. Long-term efcacy, curative potential, and carcinogenicity of topical mechloethamine chemotherapy in cutaneous T cell lymphoma. J Am Acad Dermatol 1989;20:416-428.
放疗
Yu JB, McNiff JM, Lund MW et al. Treatment of primary cutaneous CD30+ anaplastic large cell lymphoma with radiation therapy. Int J Radiat Oncol Biol Phys 2008;70:1542-1545.
全身治疗
甲氨蝶呤
Everett MA. Treatment of lymphomatoid papulosis with methotrexate. Br J Dermatol 1984;111:631.
Vonderheid EC, Sajjadian A, Kaden ME. Methotrexate is effective for lymphomatoid papulosis and other primary cutaneous CD30+ lymphoproliferative disorders. J Am Acad Dermatol 1996;34:470-481.
Fujita H, Nagatani T, Miyazawa M et al. Primary cutaneous anaplastic large cell lymphoma successfully treated with low-dose methotrexate. Eur J Dermatol 2008;18:360-361.
普拉曲沙
Horwitz SM, Kim YH, Foss F, et al. Identifcation of an active, well-tolerated dose of pralatrexate in patients with relapsed or refractory cutaneous T cell lymphoma. Blood 2012;119:4115-4122.
全身类视黄醇
Nakamura S, Hashimoto Y, Nishi K, et al. Primary cutaneous CD30+ lymphoproliferative disorder successfully treated with etretinate. Eur J Dermatol 2012;22:709-710. Krathen RA, Ward S, Duvic M. Bexarotene is a new treatment option for lymphomatoid papulosis. Dermatology 2003;206:142-147.
Wyss M, Dummer R, Dommann SN, et al. Lymphomatoid papulosis: treatment with recombinant interferon alfa-2a and etretinate. Dermatology 1995;190:288-291.
Sheehy JM, Catherwood M, Pettengeil R, et al. Sustained response of primary cutaneous CD30+ anaplastic large cell lymphoma to bexarotene and photopheresis. Leuk Lymphoma 2009;50:1389-1391.
干扰素
Proctor SJ, Jackson GH, Lennard AL, et al. Lymphotoid papulosis: response to treatment with recombinant interferon alfa-2b. J Clin Oncol 1992;10:170.
Yagi H, Tokura Y, Furukawa F, et al. Th2 cytokine mRNA expression in primary cutaneous CD30+ lymphoproliferative disorders: successful treatment with recombinant interferongamma. J Invest Dermatol 1996;107:827-832.
Schmuch M, Topar G, Illersperger B, et al. Therapeutic use of interferon-alpha for lymphomatoid papulosis. Cancer 2000;89:1603-1610.
Brentuximab vedotin
Duvic M, Tetzlaff MT, 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;33:3759-65. Broccoli A, Derenzini E, Pellegrini C, et al. Complete response of relapsed systemic and cutaneous anaplastic large cell lymphoma using brentuximab vedotin: 2 case reports. Clin Lymphoma Myeloma Leuk 2013;13:493-495.
Mody K, Wallace JS, Stearns DM, et al. CD30+ cutaneous T cell lymphoma and response to brentuximab vedotin: 2 illustrative cases. Clin Lymphoma Myeloma Leuk 2014;13:319-323. Desai A, Telang GH, Olszewski AJ. Remission of primary cutaneous anaplastic large cell lymphoma after a brief course of brentuximab vedotin. Ann Hematol 2013;92:567-568.
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