4例原发性皮肤弥漫大B细胞淋巴瘤腿型报道并文献复习

Primary Cutaneous Diffuse Large B-cell Lymphoma,Leg Type:A Report of 4 Cases with Literature Review

目的:分析原发性皮肤弥漫大B细胞淋巴瘤腿型(PCLBCL,leg type)的发病率、发病年龄、临床特点、转移规律、治疗方法及影响预后的因素。方法:收集1970年1月至2010年9月间收治的4例PCLBCL,leg type患者的资料,结合性别、年龄、首发部位(腿型或非腿型)、病变范围、乳酸脱氢酶(LDH)、β2-微球蛋白(β2-MG)、B症状、复发情况、转移部位、治疗方法、生存期等分析预后相关因素。结果:1970年1月至2010年9月间天津市平均人口数中腿型PCLBCL的发病率为4.8/1 000万。4例患者中男2例,女2例;年龄2～55岁,中位年龄29岁。临床表现多为大小不一的结节或肿块。首发部位:腿型3例,非腿型(头颈部)1例。病变范围:局限性病变者3例,弥漫性病变者1例。随访显示1例出现淋巴结受累。采用手术+化疗3例,单纯手术1例。化疗主要采用CHOP或CHOP样方案。中位生存时间仅为30.5个月。男、女患者平均生存期分别为25、219.5个月,腿型、非腿型平均生存期分别为162、3个月。局限性、弥漫性病变平均生存期分别为158、14个月,复发(是、否)平均生存期分别为30.5、214个月。结论:腿型PCLBCL是一种罕见的结外淋巴瘤,临床症状不典型,确诊有赖于组织病理学检查,治疗推荐以手术+化疗为主的综合治疗。预后可能与性别、首发部位、病变范围、有无复发有关。

Objective： To explore the morbidity, clinical features, metastasis, treatment, and prognostic factors of primary cutaneous diffuse large B-cell lymphoma, leg type （ PCDLBCL ）. Methods： The clinical records of 4 patients treated at the Tianjin Medical University Cancer Hospital from January 1970 to September 2010, including gender, age, site of cutaneous involvement （ leg or nonleg, extent of cutaneous involvement, lactate dehydrogenase （ LDH, 132-microglobulin （ 132-MG ）, B symptoms, relapse （ yes orno ）, involvement of lymph nodes and organs, treatment and survival were retrospectively reviewed. Results： From January 1970 to September 2010, the incidence of PCLBCL, leg type in Tianjin was 4.8/10 million. Of the 4 patients, 2 were males and 2 were females,aged from 2 to 55 years. The median age was 29 years. The cutaneous nodules or tumors were common clinical features. Of the 4 pa- tients, 3 involved the legs and 1 was non-leg, 3 were localized involvements, and 1 was disseminated involvement. Lymph nodes （ 1case ） were involved in follow-up visit. Three patients underwent a surgical operation combined with chemotherapy and only 1 underwent only the surgical operation. CHOP or CHOP-like chemotherapy was predominant. The median survival time was only 30.5months. The mean survival time of the males and females was 25 and 219.5 months, respectively. The mean survival time in terms of the site of cutaneous involvement （ leg and non-leg ） were 162 and 3 months, respectively. The mean survival times in term of the extent of cutaneous involvement （ localized and disseminated ） were 158 and 14 months, respectively. The mean survival time in terms of relapse （ yes, no ） were 30.5 and 214 months, respectively. Conclusion： PCLBCL, leg type is a rare extranodal lymphoma. The clinicalmanifestations are atypical. The diagnosis of PCLBCL, leg type is confirmed by histopathologic examination. Surgical operation combined with chemotherapy could improve the prognosis, but the prognosis is still poor. The prognosis is probably associated with gender,site of cutaneous involvement, extent of cutaneous involvement, and relapse.