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原发性皮肤滤泡中心淋巴瘤弥漫型早期病变的临床病理特征:对早期诊断和治疗的影响。

Clinicopathologic features of early lesions of primary cutaneous follicle center lymphoma, diffuse type: implications for early diagnosis and treatment.

机构信息

Research Unit of Dermatopathology, Department of Dermatology, Medical University of Graz, Graz, Austria.

出版信息

J Am Acad Dermatol. 2011 Nov;65(5):991-1000. doi: 10.1016/j.jaad.2010.06.059. Epub 2011 Jun 24.

Abstract

BACKGROUND

Data on early lesions of primary cutaneous follicle center lymphoma (PCFCL), diffuse type are very limited.

OBJECTIVE

We sought to elucidate the early clinicopathologic features of PCFCL, diffuse type.

METHODS

Clinical, histologic, immunohistologic, molecular, and fluorescence in situ hybridization data from 24 patients with early lesions of PCFCL, diffuse type (male:female = 19:5; median age: 57 years) were determined.

RESULTS

Lesions consisted mostly of solitary or clustered papules and small nodules located on the trunk (21 cases), arm (two cases), and scalp (one case). In 3 patients small papules were located at a distance from the main affected area. All biopsy specimens from early lesions showed aggregates of medium and large centrocytes admixed with small lymphocytes without formation of clear-cut lymph follicles. Staining for Bcl-2 was positive in only 7 cases, one revealing also a rearranged BCL2 signal by fluorescence in situ hybridization. Data on treatment and follow-up were available for 22 patients. At last examination 13 patients were in complete remission (median follow-up: 60 months), 6 were alive with skin disease alone (median follow-up: 60 months), two were alive with skin disease and bone-marrow or lymph node involvement, respectively, and one died of unrelated causes while in complete remission.

LIMITATIONS

The retrospective study and the fact that patients were treated at different institutions are limitations.

CONCLUSIONS

Early lesions of PCFCL, diffuse type present with characteristic clinicopathologic features. Dermatologists should be alert particularly to the early clinical manifestations of this lymphoma and to the presence of small, inconspicuous lesions at a distance from the main affected area in order to plan treatment properly.

摘要

背景

原发性皮肤滤泡中心淋巴瘤(PCFCL)早期病变的数据非常有限。

目的

我们旨在阐明弥漫型 PCFCL 的早期临床病理特征。

方法

从 24 例弥漫型 PCFCL 早期病变患者(男:女=19:5;中位年龄:57 岁)中确定了临床、组织学、免疫组织化学、分子和荧光原位杂交数据。

结果

病变主要由位于躯干(21 例)、手臂(2 例)和头皮(1 例)的孤立或聚集性丘疹和小结节组成。在 3 例患者中,小丘疹位于远离主要受累区域的部位。所有早期病变的活检标本均显示中等和大中心细胞的聚集,混合有小淋巴细胞,没有形成明确的淋巴滤泡。仅 7 例 Bcl-2 染色阳性,1 例通过荧光原位杂交显示 BCL2 信号重排。22 例患者可获得治疗和随访数据。最后一次检查时,13 例患者完全缓解(中位随访:60 个月),6 例患者皮肤疾病存活(中位随访:60 个月),2 例患者分别在皮肤疾病和骨髓或淋巴结受累时存活,1 例因无关原因死亡而处于完全缓解状态。

局限性

本研究为回顾性研究,且患者在不同机构接受治疗,这是本研究的局限性。

结论

弥漫型 PCFCL 的早期病变具有特征性的临床病理特征。皮肤科医生应特别注意这种淋巴瘤的早期临床表现,以及在远离主要受累区域存在小而不明显的病变,以便适当计划治疗。

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