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首页> 外文期刊>Blood: The Journal of the American Society of Hematology >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.
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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.

机译：EORTC，ISCL和USCLC对于治疗原发性皮肤CD30阳性的淋巴增生性疾病的共识性建议：淋巴瘤样丘疹和原发性皮肤间变性大细胞淋巴瘤。

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摘要

Primary cutaneous CD30(+) lymphoproliferative disorders (CD30(+) LPDs) are the second most common form of cutaneous T-cell lymphomas and include lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. Despite the anaplastic cytomorphology of tumor cells that suggest an aggressive course, CD30(+) LPDs are characterized by an excellent prognosis. Although a broad spectrum of therapeutic strategies has been reported, these have been limited mostly to small retrospective cohort series or case reports, and only very few prospective controlled or multicenter studies have been performed, which results in a low level of evidence for most therapies. The response rates to treatment, recurrence rates, and outcome have not been analyzed in a systematic review. Moreover, international guidelines for staging and treatment of CD30(+) LPDs have not yet been presented. Based on a literature analysis and discussions, recommendations were elaborated by a multidisciplinary expert panel of the Cutaneous Lymphoma Task Force of the European Organization for Research and Treatment of Cancer, the International Society for Cutaneous Lymphomas, and the United States Cutaneous Lymphoma Consortium. The recommendations represent the state-of-the-art management of CD30(+) LPDs and include definitions for clinical endpoints as well as response criteria for future clinical trials in CD30(+) LPDs.
机译：原发性皮肤CD30（+）淋巴增生性疾病（CD30（+）LPD）是皮肤T细胞淋巴瘤的第二大最常见形式，包括淋巴瘤样丘疹和原发性皮肤间变性大细胞淋巴瘤。尽管肿瘤细胞的变性细胞形态学表明其具有侵袭性，但CD30（+）LPD的特征是预后良好。尽管已报道了广泛的治疗策略，但这些策略主要限于小型回顾性队列研究或病例报告，并且仅进行了极少的前瞻性对照或多中心研究，导致大多数疗法的证据水平较低。对治疗的反应率，复发率和结果尚未进行系统的分析。此外，尚未提出关于CD30（+）LPD的分期和治疗的国际指南。在文献分析和讨论的基础上，欧洲癌症研究与治疗组织皮肤淋巴瘤工作组，国际皮肤淋巴瘤学会和美国皮肤淋巴瘤协会的多学科专家小组详细阐述了建议。这些建议代表了CD30（+）LPD的最新管理，并包括临床终点的定义以及CD30（+）LPD未来临床试验的反应标准。

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