CD30+ cutaneous lymphoproliferative disorders:: The Stanford experience in lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma
CD30+ cutaneous lymphoproliferative disorders:: The Stanford experience in lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma
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DOI：
10.1016/s0190-9622(03)02484-8
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发表时间：
2003-12-01
影响因子：
13.8
通讯作者：
Kim, YH
中科院分区：
文献类型：
--
作者：
Liu, HL;Hoppe, RT;Kim, YH
背景资料：CD 30（+）皮肤淋巴增生性疾病（CLPD）包括淋巴瘤样丘疹病、CD 30（+）CLPD的临界病例和原发性皮肤间变性大细胞淋巴瘤（PCALCL）。先前的研究表明CD 30（+）CLPDs具有良好的预后。目的：我们试图介绍斯坦福大学，斯坦福大学，加利福尼亚州，在CD 30（+）CLPDs的管理方面的单中心经验。方法：对在我们机构治疗的56例CD 30（+）CLPDs患者进行回顾性队列分析。结果：无淋巴瘤样丘疹病患者死于疾病，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%。局限性与泛发性PCALCL的5年疾病特异性生存率分别为91%和50%（P = 0.31）。PCALCL对治疗有高度反应，但复发率为42%。共有3例患者进展至皮外期。没有分析的临床或组织学因素预测淋巴瘤样丘疹病和PCALCL的预后较差。与欧洲多中心研究组的既往报告相似，我们机构的单中心经验表明CD 30（+）CLPD具有总体良好的预后;然而，确实存在预后不良的PCALCL病例。
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.