皮肤原发性血管纤维黏液性T细胞淋巴瘤一例

目的 探讨皮肤原发性T细胞淋巴瘤一些新的临床病理亚型特点。方法 对1例具有新的临床病理特点的T细胞淋巴瘤多次活检、多种病理方法检查及临床资料综合分析,包括常规H-E染色、特殊染色、免疫组化染色、基因重排及PCR检测。结果 皮损为柔软的多发结节,并有黏液囊肿形成;肿瘤细胞以小T淋巴细胞为主,主要浸润真皮及皮下,无明显亲表皮现象。部分肿瘤细胞有亲血管现象,伴明显血管纤维黏液样基质。免疫组化及特殊染色结果显示,CD3,CD43,CD45RO均阳性,CD56少数阳性;CD68,CD79α,CD20,CD30,CD117,ALK,S-100,CD45R,EMA,SMA均阴性;黏液基质阿新蓝染色阳性;基因重排TCR-β阳性;PCR检测EBV病毒阴性。结论 伴血管纤维黏液样基质的T细胞淋巴瘤是皮肤原发性T细胞淋巴瘤一种新的、独特的亚型,不属于亲表皮的蕈样肉芽肿亚型。

Fibromucinous Primary Cutaneous T-Cell Lymphoma Rich in Blood Vessels: A Case Report

Objective To study a new clinicopathological subtype of primary cutaneous T-cell lym phoma (PCTCL). Methods A case of T-cell lymphoma was systematically evaluated clinically and by using H-E staining, special staining,immunohistochemical staining,gene rearrangement and PCR. Results The skin lesion presented as tender nodules with mucocele. Skin biopsies showed that in the dermis and subcutaneous tissue,most of the angiotropic tumor cells were small T cells; no obvious epidermotropic phenomenon was detected.A few vessels were observed with obvious fibromucinous matrix formation. Immunohislochemical studies showed the following:CD3(+),CD43(+),CD45RO(+),CD56(a few),CD68(-), CD79α(-),CD20 (-), CD30(-), CD117(-), ALK(-), S-100(-),CD45R(-),EMA(-),SMA(-).The mucoid matrix was positive for Alcian blue staining.The rearrangement of T-cell β receptor gene was detected.EBV was not detected with PCR. Conclusion Fibromucinous T-cell lymphoma rich in blood vessels is a new and distinct variant of PCTCL; it is not a subtype of mycosis fungoides.