Paulli M, Berti E, Rosso R, Boveri E, Kindl S, Klersy C, Lazzarino M, Borroni G, Menestrina F, Santucci M
Institute of Pathology, Biometry, Hematology and Dermatology, I.R.C.C.S. Policlinico S. Matteo.
J Clin Oncol. 1995 Jun;13(6):1343-54. doi: 10.1200/JCO.1995.13.6.1343.
Recently, it has been shown that CD30 antigen expression is associated with a relatively favorable prognosis in primary cutaneous large-cell lymphomas (CLCLs). However, prognostic subsets within the CD30+ group have been difficult to identify due to lack of uniform clinicopathologic and immunophenotypic criteria, limited clinical information, and the inclusion of relatively few patients for statistical analysis in prior studies. To address these problems, we formed a multicentric study group of pathologists and dermatologists to classify and evaluate 92 cases of CD30+ cutaneous lymphoproliferative disorders.
An expert panel established consensus diagnoses for 86 CD30+ cutaneous lymphomas. Cases, clinically and histologically classified as lymphomatoid papulosis (LyP), anaplastic large-cell lymphoma (ALCL), nonanaplastic lymphoma, and borderline histology between LyP and ALCL, were then analyzed statistically by univariate, multivariate, and Cox regression model analysis of potential prognostic features.
Spontaneous regression and age less than 60 years were associated with a favorable prognosis, while extracutaneous disease and age greater than 60 had a poor prognosis. Patients with LyP had the best prognosis, followed by those with primary CD30+ lymphomas, regardless of cytologic type (anaplastic or nonanaplastic). Borderline cases, morphologically indistinguishable from LyP and CD30+ ALCL, had a favorable prognosis, similar to LyP.
Our findings indicate that CD30+ cutaneous lymphoproliferative disorders comprise a spectrum of closely related skin lesions, which can be assigned a relatively favorable or unfavorable prognosis by a combined clinical and pathologic analysis.
最近研究表明,CD30抗原表达与原发性皮肤大细胞淋巴瘤(CLCL)相对良好的预后相关。然而,由于缺乏统一的临床病理和免疫表型标准、临床信息有限以及既往研究纳入的用于统计分析的患者相对较少,CD30阳性组内的预后亚组难以确定。为解决这些问题,我们组建了一个由病理学家和皮肤科医生组成的多中心研究小组,对92例CD30阳性皮肤淋巴增殖性疾病进行分类和评估。
一个专家小组对86例CD30阳性皮肤淋巴瘤做出了共识诊断。然后,对临床和组织学分类为淋巴瘤样丘疹病(LyP)、间变性大细胞淋巴瘤(ALCL)、非间变性淋巴瘤以及LyP和ALCL之间临界组织学的病例,通过对潜在预后特征进行单变量、多变量和Cox回归模型分析进行统计学分析。
自发消退和年龄小于60岁与良好的预后相关,而皮肤外疾病和年龄大于60岁预后较差。LyP患者预后最佳,其次是原发性CD30阳性淋巴瘤患者,无论细胞类型(间变性或非间变性)如何。临界病例在形态上与LyP和CD30阳性ALCL无法区分,其预后良好,与LyP相似。
我们的研究结果表明,CD30阳性皮肤淋巴增殖性疾病包括一系列密切相关的皮肤病变,通过临床和病理联合分析可判断其预后相对良好或不良。