原发性皮肤CD30阳性大细胞淋巴瘤：一种预后良好的新型皮肤淋巴瘤的定义。一项对47例患者的欧洲多中心研究。

Primary cutaneous CD30-positive large cell lymphoma: definition of a new type of cutaneous lymphoma with a favorable prognosis. A European Multicenter Study of 47 patients.

作者信息

Beljaards R C, Kaudewitz P, Berti E, Gianotti R, Neumann C, Rosso R, Paulli M, Meijer C J, Willemze R

机构信息

Department of Dermatology, Free University Hospital of Amsterdam, The Netherlands.

出版信息

Cancer. 1993 Mar 15;71(6):2097-104. doi: 10.1002/1097-0142(19930315)71:6<2097::aid-cncr2820710626>3.0.co;2-7.

DOI:10.1002/1097-0142(19930315)71:6<2097::aid-cncr2820710626>3.0.co;2-7

PMID:8382999

Abstract

BACKGROUND

CD30 (Ki-1)-positive anaplastic large cell lymphoma (LCL) has been described as a morphologically distinct group of LCL that generally are associated with a poor prognosis. Recent studies indicate that these lymphomas, when confined to the skin, have a favorable prognosis. However, there is no consensus regarding the definition of these primary cutaneous CD30-positive LCL. Reported patients have been selected variously on the basis of morphologic (anaplastic cytology) or immunophenotypical (expression of CD30 antigen) criteria.

METHODS

At two recent workshops aimed to achieve consensus on the definition and terminology of these lymphomas, the clinical, histologic, and immunophenotypical data of 47 patients with primary cutaneous CD30-positive LCL from five collaborating European centers were analyzed.

RESULTS

Characteristic clinical features were presentation with solitary or localized skin lesions (42 of 47 patients), frequent cutaneous relapses (15 patients), and partial or complete spontaneous remission of skin lesions (11 patients). Twelve of 47 (25%) patients developed extracutaneous disease. The favorable prognosis of these lymphomas is indicated by the follow-up data that show that 36 of 47 patients are alive and in complete remission, only four disease-related deaths have occurred, and the overall median survival is 42 months (range, 2-130 months). There were no differences in clinical presentation, course, or prognosis between anaplastic and nonanaplastic CD30-positive LCL.

CONCLUSION

The results of this study indicate that primary cutaneous CD30-positive LCL, regardless of their morphologic classification (anaplastic or nonanaplastic) can be considered as a distinct type of cutaneous T-cell lymphoma. Recognition of this type of cutaneous lymphoma is important because it may prevent patients from unnecessary aggressive treatment.

摘要

背景

CD30（Ki-1）阳性间变性大细胞淋巴瘤（LCL）被描述为一组形态学上独特的LCL，通常预后较差。最近的研究表明，这些淋巴瘤局限于皮肤时预后良好。然而，对于这些原发性皮肤CD30阳性LCL的定义尚无共识。报道的患者是根据形态学（间变性细胞学）或免疫表型（CD30抗原表达）标准以不同方式选择的。

方法

在最近两次旨在就这些淋巴瘤的定义和术语达成共识的研讨会上，分析了来自五个欧洲合作中心的47例原发性皮肤CD30阳性LCL患者的临床、组织学和免疫表型数据。

结果

特征性临床特征为出现孤立性或局限性皮肤病变（47例患者中的42例）、频繁皮肤复发（15例患者）以及皮肤病变部分或完全自发缓解（11例患者）。47例患者中有12例（25%）发生皮肤外疾病。这些淋巴瘤的良好预后通过随访数据表明，47例患者中有36例存活且完全缓解，仅发生4例与疾病相关的死亡，总体中位生存期为42个月（范围为2 - 130个月）。间变性和非间变性CD30阳性LCL在临床表现、病程或预后方面无差异。