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两例胸壁原发性皮肤弥漫大 B 细胞淋巴瘤,腿型累及骨骼。

Bone involvement in two cases of thoracic primary cutaneous diffuse large B-cell lymphoma, leg type.

机构信息

Department of Dermatology, Tenon Hospital, Paris, France.

出版信息

Eur J Dermatol. 2011 Sep-Oct;21(5):744-9. doi: 10.1684/ejd.2011.1431.

DOI:10.1684/ejd.2011.1431
PMID:21700538
Abstract

Primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL-LT), is defined by a predominance of confluent sheets of centroblasts and immunoblasts, which strongly express Bcl-2 protein. This cutaneous lymphoma is mainly characterized by the development of skin lesions on the lower leg. Other localizations are possible (namely non-leg PCLBCL-LT) and usually affect younger patients. PCLBCL-LT is distinguished from the two other subtypes of primary cutaneous B-cell lymphomas by its immunohistopathological features, its aggressive clinical behaviour with a worse prognosis linked to skin recurrences, but also secondary extracutaneous spread. Bone involvement underlying skin lesions has been reported in few series and cases reports during PCLBCL-LT. We describe here two aggressive cases of PCLBCL-LT with high burden and infiltrative thoracic tumours, with localized bone involvement.

摘要

原发性皮肤弥漫性大 B 细胞淋巴瘤,腿型(PCLBCL-LT)的特征是中胚层母细胞和免疫母细胞的融合片明显增多,强烈表达 Bcl-2 蛋白。这种皮肤淋巴瘤主要表现为小腿皮肤损伤。其他部位也可能发生病变(即非腿 PCLBCL-LT),且通常影响年轻患者。PCLBCL-LT 通过其免疫组织病理学特征与原发性皮肤 B 细胞淋巴瘤的另外两种亚型相区别,其侵袭性临床表现与皮肤复发相关,预后较差,但也可能继发皮肤外播散。在少数系列和病例报告中曾报道过 PCLBCL-LT 皮肤病变下的骨受累。我们在此描述了两例侵袭性 PCLBCL-LT 病例,它们的胸内肿瘤负荷高且浸润性强,局部有骨受累。

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