Liu Q, Ohshima K, Kikuchi M
Department of Pathology, School of Medicine, Fukuoka University, Fukuoka, Japan.
Pathol Int. 2000 Dec;50(12):960-6. doi: 10.1046/j.1440-1827.2000.01151.x.
Primary cutaneous B-cell lymphoma (PCBCL) is a rare group of lymphoproliferative disorders. There have been few reports of Japanese patients with PCBCL, so the present study investigated the clinicopathological and immunological features and Bcl-2 gene rearrangement and protein expression in 28 Japanese patients with PCBCL. According to the Revised European-American Lymphoma (REAL) classification, there were 25 diffuse large B-cell lymphomas (DLBCL), one Burkitt type lymphoma, one lymphoblastic lymphoma and one marginal zone cell lymphoma. Of the 25 DLBCL, 17 were in males and eight in females, with an average age of 69.4 years. Follow-up data were available in 19 cases of DLBCL of which seven died and 12 were alive. The overall 5-year survival rate was 61%. Cases of DLBCL involving the legs were found to have poorer clinical outcomes; two of four cases with leg lesions died, with a mean survival of 13 months. Of 14 cases with non-leg lesions, four died, and the mean survival was 38.9 months. Only one case of Burkitt type lymphoma was CD10 positive. Bcl-2 rearrangement was not observed in 13 cases studied by polymerase chain reaction. Bcl-2 expression was observed in nine of 13 cases studied. All five cases with leg lesions exhibited Bcl-2 expression, but four of six cases with non-leg lesions also expressed the protein. These results show that DLBCL is the most frequent subtype of PCBCL in Japanese patients and that the prognosis of Japanese patients with DLBCL is worse than that of reported European cases. The study also found that PCBCL was frequently associated with Bcl-2 expression, which was not site-confined, and that there was no evidence for a follicular center origin of PCBCL.
原发性皮肤B细胞淋巴瘤（PCBCL）是一组罕见的淋巴增殖性疾病。关于日本PCBCL患者的报道较少，因此本研究调查了28例日本PCBCL患者的临床病理和免疫学特征、Bcl-2基因重排及蛋白表达情况。根据修订的欧美淋巴瘤（REAL）分类，其中有25例弥漫性大B细胞淋巴瘤（DLBCL）、1例伯基特型淋巴瘤、1例淋巴母细胞淋巴瘤和1例边缘区细胞淋巴瘤。在25例DLBCL中，男性17例，女性8例，平均年龄69.4岁。19例DLBCL有随访数据，其中7例死亡，12例存活。总体5年生存率为61%。发现累及腿部的DLBCL病例临床结局较差；4例腿部病变患者中有2例死亡，平均生存期为13个月。14例非腿部病变患者中，4例死亡，平均生存期为38.9个月。仅1例伯基特型淋巴瘤CD10呈阳性。通过聚合酶链反应研究的13例病例中未观察到Bcl-2重排。在研究的13例病例中有9例观察到Bcl-2表达。所有5例腿部病变患者均表现出Bcl-2表达，但6例非腿部病变患者中有4例也表达该蛋白。这些结果表明，DLBCL是日本PCBCL患者中最常见的亚型，且日本DLBCL患者的预后比报道的欧洲病例更差。该研究还发现，PCBCL常与Bcl-2表达相关，且这种表达不受部位限制，并且没有证据表明PCBCL起源于滤泡中心。
