Department of Dermatology, Hospital Universitari de Bellvitge, IDIBELL, Barcelona, Spain.
J Am Acad Dermatol. 2013 Sep;69(3):357-65. doi: 10.1016/j.jaad.2013.04.047. Epub 2013 Jun 21.
Primary cutaneous marginal zone B-cell lymphomas are low-grade lymphomas running an indolent course. Skin relapses have been frequently reported but little information about disease-free survival (DFS) is available.
We sought to evaluate relapse rate and DFS in patients with primary cutaneous marginal zone B-cell lymphomas.
Clinical features, European Organization for Research and Treatment of Cancer/International Society for Cutaneous Lymphomas stage, light chain restriction, clonality, treatments, skin relapses, DFS, stage progression, extracutaneous disease, and outcome are analyzed in a series of 137 patients.
Patients were classified as solitary lesion (T1) (n = 70; 51%), regional skin involvement (T2) (n = 40; 29%), and generalized skin lesions (T3) (n = 27; 20%). Surgical excision, local radiotherapy, or a combination were the initial treatment in 118 patients (86%). In 121 of 137 patients (88%) a complete remission was observed after initial treatment, including 99 of 106 patients (93%) with solitary or localized disease and 22 of 31 patients (71%) with multifocal lesions. Cutaneous relapses were observed in 53 patients (44%). Median DFS was 47 months. Patients with multifocal lesions or T3 disease showed higher relapse rate and shorter DFS. No significant differences were observed between surgery and radiotherapy, but surgery alone was associated with more recurrences at initial site. Overall survival at 5 and 10 years was 93%. Six patients (4%) developed extracutaneous disease during follow-up.
This was a case series retrospective study.
Our results support long-term follow-up in patients with primary cutaneous marginal zone B-cell lymphomas. Disseminated skin lesions have higher relapse rate and shorter DFS suggesting further investigation on systemic therapies in such a group of patients.
原发性皮肤边缘区 B 细胞淋巴瘤为低级别淋巴瘤，呈惰性病程。皮肤复发较为常见，但关于无疾病生存（DFS）的数据较少。
我们旨在评估原发性皮肤边缘区 B 细胞淋巴瘤患者的复发率和 DFS。
我们分析了 137 例患者的临床特征、欧洲癌症研究与治疗组织/国际皮肤淋 巴瘤研究协会分期、轻链限制、克隆性、治疗方法、皮肤复发、DFS、分期进展、皮肤外疾病和预后。
患者分为孤立性病变（T1）（n=70；51%）、区域性皮肤受累（T2）（n=40；29%）和全身性皮肤病变（T3）（n=27；20%）。118 例患者（86%）接受了手术切除、局部放疗或联合治疗作为初始治疗。137 例患者中，121 例（88%）在初始治疗后观察到完全缓解，包括 99 例（93%）孤立性或局限性疾病患者和 22 例（71%）多发性病变患者。53 例（44%）患者发生皮肤复发。中位 DFS 为 47 个月。多发性病变或 T3 疾病患者的复发率更高，DFS 更短。手术与放疗之间无显著差异，但单独手术与初始部位的更多复发相关。5 年和 10 年的总生存率分别为 93%。6 例（4%）患者在随访期间发生皮肤外疾病。
这是一项回顾性病例系列研究。
我们的结果支持对原发性皮肤边缘区 B 细胞淋巴瘤患者进行长期随访。播散性皮肤病变的复发率更高，DFS 更短，提示在这组患者中进一步研究全身性治疗。
