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16 岁女孩原发性皮肤滤泡中心淋巴瘤。

Primary cutaneous follicle center lymphoma in a 16-year-old girl.

机构信息

Department of Pathology, University of Virginia, Charlottesville, Virginia, USA.

Department of Pathology, Inform Diagnostics, Boston, Massachusetts, USA.

出版信息

J Cutan Pathol. 2021 May;48(5):663-668. doi: 10.1111/cup.13939. Epub 2021 Jan 13.

Abstract

In the pediatric and adolescent age group, primary cutaneous lymphomas are rare, especially cutaneous B-cell lymphomas. According to the World Health Organization, the three main subtypes of primary cutaneous B-cell lymphomas are primary cutaneous marginal zone B-cell lymphoma (PCMZL), primary cutaneous follicle center lymphoma (PCFCL), and primary cutaneous diffuse large B-cell lymphoma, leg type. We present an extraordinary case of PCFCL arising in a 16-year-old female, only the sixth case of PCFCL to be reported in the literature in a patient less than 20 years old. Although PCMZL was considered in this case, the finding of lambda light chain restriction in the BCL-6 and CD10 positive population of lymphocytes established the diagnosis of primary cutaneous follicle center lymphoma. Not many data currently exist on the prognosis of PCFCL in young individuals, but adult PCFCL has a good prognosis with an indolent course and 5-year survival rates over 95%. Because of its uncommon manifestation in young patients, the diagnosis of PCFCL is often delayed or missed. This case is presented to raise awareness of PCFCL in the pediatric/ adolescent population and to contribute to the ongoing research of this condition.

摘要

在儿科和青少年群体中,原发性皮肤淋巴瘤较为罕见,尤其是皮肤 B 细胞淋巴瘤。根据世界卫生组织的分类,原发性皮肤 B 细胞淋巴瘤主要包括原发性皮肤边缘区 B 细胞淋巴瘤(PCMZL)、原发性皮肤滤泡中心淋巴瘤(PCFCL)和原发性皮肤弥漫性大 B 细胞淋巴瘤,腿型。我们报告了一例发生在 16 岁女性的特殊 PCFCL 病例,这是文献中第六例报道的年龄小于 20 岁的 PCFCL 病例。尽管在该病例中考虑了 PCMZL,但 BCL-6 和 CD10 阳性淋巴细胞中存在 lambda 轻链限制的发现确立了原发性皮肤滤泡中心淋巴瘤的诊断。目前关于年轻个体中 PCFCL 的预后数据不多,但成人 PCFCL 具有良好的预后,呈惰性病程,5 年生存率超过 95%。由于其在年轻患者中的表现不常见,因此 PCFCL 的诊断常常被延迟或遗漏。本病例旨在提高对儿科/青少年人群中 PCFCL 的认识,并为该疾病的持续研究做出贡献。

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