Grange Florent, Beylot-Barry Marie, Courville Phillipe, Maubec Eve, Bagot Martine, Vergier Béatrice, Souteyrand Pierre, Machet Laurent, Dalac Sophie, Esteve Eric, Templier Isabelle, Delaporte Emmanuel, Avril Marie-Françoise, Robert Caroline, Dalle Stephane, Laroche Liliane, Delaunay Michele, Joly Pascal, Wechsler Janine, Petrella Tony
Department of Dermatology, Hôpital Robert Debré, Avenue du Général Koenig, Reims 51100, France.
Arch Dermatol. 2007 Sep;143(9):1144-50. doi: 10.1001/archderm.143.9.1144.
To describe clinicopathologic features and to identify prognostic factors in a large series of primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL LT), as defined in the recent World Health Organization-European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas.
Retrospective multicenter study from the French Study Group on Cutaneous Lymphomas.
Nineteen departments of dermatology in 10 regions of France.
Sixty patients with a PCLBCL LT included in the registry of the French Study Group on Cutaneous Lymphomas.
Age, sex, outcome, therapy, B symptoms, cutaneous extent, number of lesions, location (leg vs nonleg), serum lactate dehydrogenase level, and MUM-1 and Bcl-2 expression were recorded. Disease-specific survival was used as the main end point. Prognostic factors were identified using a Cox proportional hazards model.
Primary cutaneous diffuse large B-cell lymphoma, leg type is characterized by a predilection for the leg (72%), a high proportion of Bcl-2 expression (85%), an advanced age at onset (mean age, 76 years), and frequent relapses and extracutaneous dissemination. The overall 5-year disease-specific survival rate was 41%. Location on the leg and multiple skin lesions were predictive of death in multivariate analysis. Although no variable related to therapy was significantly associated with survival, patients recently treated with combinations of anthracycline-containing chemotherapies and rituximab had a more favorable short-term outcome.
Primary cutaneous diffuse large B-cell lymphoma, leg type is a distinct entity with a poor prognosis, particularly in patients with multiple tumors on the legs. Despite the advanced age of many patients, the prognosis could be improved with combinations of anthracycline-containing chemotherapies and rituximab.
描述大量原发性皮肤弥漫性大B细胞淋巴瘤（腿部型，PCLBCL LT）的临床病理特征，并确定预后因素，该疾病定义参照世界卫生组织-欧洲癌症研究与治疗组织最近的皮肤淋巴瘤分类。
法国皮肤淋巴瘤研究组开展的一项回顾性多中心研究。
法国10个地区的19个皮肤科。
60例腿部型原发性皮肤弥漫性大B细胞淋巴瘤患者纳入法国皮肤淋巴瘤研究组登记系统。
记录年龄、性别、预后、治疗、B症状、皮肤累及范围、皮损数量、部位（腿部与非腿部）、血清乳酸脱氢酶水平以及MUM-1和Bcl-2表达情况。以疾病特异性生存率作为主要终点。采用Cox比例风险模型确定预后因素。
原发性皮肤弥漫性大B细胞淋巴瘤腿部型的特点为好发于腿部（72%）、Bcl-2高表达比例（85%）、发病年龄较大（平均年龄76岁）以及频繁复发和皮肤外播散。5年总体疾病特异性生存率为41%。多因素分析显示，腿部发病和多发皮肤损害可预测死亡。尽管没有与治疗相关的变量与生存率显著相关，但近期接受含蒽环类化疗药物与利妥昔单抗联合治疗的患者短期预后较好。
原发性皮肤弥漫性大B细胞淋巴瘤腿部型是一种预后较差的独特疾病，尤其是腿部有多个肿瘤的患者。尽管许多患者年龄较大，但含蒽环类化疗药物与利妥昔单抗联合治疗可能改善预后。
