原发性皮肤边缘区淋巴瘤1例

Primary cutaneous marginalzone lymphoma:A case report

患者男,41岁,背部出现皮损,不伴痛痒6个月余。皮肤科情况:背部可见一直径约1.0 cm红色、近圆形、表面光滑的结节,周围有红斑晕,质地略硬,无触压痛。组织病理检查:肿瘤细胞在真皮内弥漫性浸润,呈现上方浸润多且范围广,下方浸润少且范围窄,伴有反应性生发中心;可见“三相”模式,即中央为淡染的生发中心,绕以深染的滤泡套细胞,以及淡染的肿瘤性边缘区细胞;并可见较多浆细胞、淋巴浆细胞样细胞、边缘区细胞(较大且淡染的细胞)以及小的反应性淋巴细胞,偶有大的母细胞。免疫组化染色:肿瘤性边缘区细胞CD20、CD79a、B细胞淋巴瘤-2(Bcl-2)、多发性骨髓瘤癌基因(MUM-1)、CD43均阳性,Kappa(浆样细胞阴性),Lambda(浆样细胞阳性),Ki-67达25%;CD3、CD21、Bcl-6均阴性。分子病理检测结果示T淋巴细胞克隆性基因重排检测阴性,B细胞淋巴瘤克隆性基因重排检测阳性。诊断:原发性皮肤边缘区淋巴瘤。

A 41 year-old man presented with a skin lesion on the upper back for more than half a year,which demonstrated with no pain and itch.Physical examination showed one approximately 1.0 cm in diameter,red,smooth surfaced,slightly-hard nodule around with a focal perilesional,erythematous halo.The histopathology showed the tumor cells were infiltrated diffusely in the dermis,showing more and wider infiltration in the upper part and less and narrow infiltration in the lower part,and with reactive germinal centers,"triphasic"pattern characterized by pale areas in the center(corresponding to germinal centers)surrounded by dark cells of the follicular mantle and by pale neoplastic marginal zone cells,neoplastic cells representing a part of infiltrate and invariably admixed with reactive lymphocytes and with the formation of reactive germinal centers.There were plasma cells,lymphoplasmacytoid cells,marginal zone cells(larger and palercells),small reactive lymphocytes,and occasional large blasts.Immunohistochemistry results showed that CD20,CD79a,MUM-1,Bcl-2 and CD43 were positive,Kappa(plasma cells was negative),Lambda(plasma cells was positive),Ki-67 was 25%,CD3、CD21 and Bcl-6 were negative.Molecular genetics showed that monoclonal gene rearrangement was positive for B-cell lymphoma,T cell monoclonal gene rearrangement was negative.A diagnosis of primary cutaneous margnalzone lymphoma was made.