CD30+皮肤淋巴增殖性疾病：斯坦福大学在淋巴瘤样丘疹病和原发性皮肤间变性大细胞淋巴瘤方面的经验

CD30+ cutaneous lymphoproliferative disorders: the Stanford experience in lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma.

作者信息

Liu Howard L, Hoppe Richard T, Kohler Sabine, Harvell Jeff D, Reddy Sunil, Kim Youn H

机构信息

Department of Dermatology, Stanford University, 900 Blake Wilbur Drive, Stanford, CA 94305, USA.

出版信息

J Am Acad Dermatol. 2003 Dec;49(6):1049-58. doi: 10.1016/s0190-9622(03)02484-8.

DOI:10.1016/s0190-9622(03)02484-8

PMID:14639383

Abstract

BACKGROUND

CD30+ cutaneous lymphoproliferative disorders (CLPDs) include lymphomatoid papulosis, borderline cases of CD30+CLPDs, and primary cutaneous anaplastic large cell lymphoma (PCALCL). Prior studies have shown CD30+CLPDs have an excellent prognosis.

OBJECTIVE

We sought to present the single-center experience of Stanford University, Stanford, Calif, in the management of CD30+CLPDs.

METHODS

A retrospective cohort analysis of 56 patients with CD30+CLPDs treated at our institution was performed.

RESULTS

No patients with lymphomatoid papulosis died of disease, and overall survival was 92% at 5 and 10 years. Disease-specific survivals at 5 and 10 years for PCALCL were 85%. Disease-specific survival at 5 years for localized versus generalized PCALCL was 91% versus 50% (P =.31). PCALCL was highly responsive to treatment, but the relapse rate was 42%. In all, 3 patients progressed to extracutaneous stage of disease. No clinical or histologic factors analyzed were predictive of worse outcome in lymphomatoid papulosis and PCALCL.

CONCLUSION

Similar to prior reports from multicenter European groups, the single-center experience at our institution demonstrates CD30+CLPDs have an overall excellent prognosis; however, cases of PCALCL with poor outcome do exist.

摘要

背景

CD30+皮肤淋巴增殖性疾病（CLPDs）包括淋巴瘤样丘疹病、CD30+CLPDs的临界病例以及原发性皮肤间变性大细胞淋巴瘤（PCALCL）。既往研究表明，CD30+CLPDs预后良好。

目的

我们旨在介绍加利福尼亚州斯坦福市斯坦福大学的单中心经验，即对CD30+CLPDs的管理。

方法

对在我们机构接受治疗的56例CD30+CLPDs患者进行回顾性队列分析。

结果

淋巴瘤样丘疹病患者无死于疾病者，5年和10年总生存率为92%。PCALCL的5年和10年疾病特异性生存率为85%。局限性与全身性PCALCL的5年疾病特异性生存率分别为91%和50%（P = 0.31）。PCALCL对治疗高度敏感，但复发率为42%。共有3例患者疾病进展至皮肤外阶段。在淋巴瘤样丘疹病和PCALCL中，分析的所有临床或组织学因素均不能预测更差的预后。