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原发性皮肤大B细胞淋巴瘤,腿部型,局限于背部

Primary Cutaneous Large B-Cell Lymphoma, Leg Type, Localized on the Dorsum.

作者信息

Patrizi A, Raone B, Sabattini E, Gurioli C, Pileri A, D'Acunto C

机构信息

Dermatology, Department of Internal Medicine, Geriatrics and Nephrologic Diseases, University of Bologna, Bologna, Italy.

出版信息

Case Rep Dermatol. 2009 Nov 11;1(1):87-92. doi: 10.1159/000253874.

Abstract

Primary cutaneous large B-cell lymphoma, leg-type (PCLBCL-LT), is a large B-cell lymphoma primarily involving the skin. It is distinguished from the other 3 subsets of this lymphoproliferative disorder by its immunohistopathological features, configuring confluent sheets of medium-sized to large B lymphocytes with round nuclei provided with evident nucleoli, resembling centroblasts or immunoblasts, which express Bcl-6, Bcl-2. Prevalently appearing on the lower limbs, as a single or multicentric and frequently ulcerated skin nodule or plaque, PCLBCL-LT has a worse prognosis than the other large B-cell lymphomas. Moreover, the age of onset is delayed (7th decade) compared to those of the other 3 subtypes (6th decade); it presents a slight female predominance (2:1), and a higher percentage of positivity to Bcl-2. We present a 52-year-old man who showed a 2-year standing, non-ulcerated, round, 4 cm in diameter, red plaque, medially located on the dorsum. After biopsy the diagnosis of PCLBCL-LT was made on histopathological and immunohistochemical studies, the latter showing positivity to CD20, Bcl-2, and Bcl-6. After treatment with radiotherapy the patient has shown a 4.4-year follow-up free of disease.

摘要

原发性皮肤大B细胞淋巴瘤,腿部型(PCLBCL-LT),是一种主要累及皮肤的大B细胞淋巴瘤。它通过免疫组织病理学特征与这种淋巴增殖性疾病的其他3个亚组相区分,其特征为中等大小至大B淋巴细胞形成融合片层,细胞核圆形,有明显核仁,类似中心母细胞或免疫母细胞,表达Bcl-6、Bcl-2。PCLBCL-LT通常出现在下肢,表现为单个或多中心且常溃疡的皮肤结节或斑块,其预后比其他大B细胞淋巴瘤更差。此外,与其他3个亚型(第六个十年)相比,其发病年龄延迟(第七个十年);女性略占优势(2:1),Bcl-2阳性率更高。我们报告一名52岁男性,其背部内侧出现一个直径4厘米、圆形、持续2年的非溃疡性红色斑块。活检后,通过组织病理学和免疫组织化学研究确诊为PCLBCL-LT,后者显示CD20、Bcl-2和Bcl-6呈阳性。放疗治疗后,该患者已进行了4.4年的无病随访。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/51b6/2895218/4544f4e733d1/cde0001-0087-f01.jpg

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