Peñate Y, Servitje O, Machan S, Fernández-de-Misa R, Estrach M T, Acebo E, Mitxelena J, Ramón M D, Flórez A, Blanes M, Morillo M, Medina S, Bassas J, Zayas A, Espinosa P, Pérez A, Gónzalez-Romero N, Domínguez J D, Muniesa C, López Robles J, Combalia A, Yanguas I, Suh H, Polo-Rodríguez I, Bielsa I, Mateu A, Ferrer B, Descalzo M A, García-Doval I, Ortiz-Romero P L
Servicio de Dermatología, Complejo Hospitalario Universitario Insular Materno-Infantil de Gran Canaria, Las Palmas, España.
Servicio de Dermatología, Hospital Universitari Bellvitge, L' Hospitalet de Llobregat, Barcelona, España.
Actas Dermosifiliogr (Engl Ed). 2018 Sep;109(7):610-616. doi: 10.1016/j.ad.2018.03.006. Epub 2018 Apr 19.
Primary cutaneous lymphomas are uncommon. This article describes the Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV) and reports on the results from the first year.
Disease registry for patients with primary cutaneous lymphoma. The participating hospitals prospectively recorded data on diagnosis, treatment, tests, and disease stage for all patients with primary cutaneous lymphoma. A descriptive analysis was performed.
In December 2017, the registry contained data on 639 patients (60% male) from 16 university hospitals. The most common diagnoses, in order of frequency, were mycosis fungoides/Sézary syndrome (MF/SS) (348 cases, 55%), primary cutaneous B-cell lymphoma (CBCL) (184 cases, 29%), primary cutaneous CD30 T-cell lymphoproliferative disorder (CD30 CLPD) (70 cases, 11%), and other types of T-cell lymphoma (37 cases, 5%). In total, 105 (16.5%) of the cases recorded were incident cases. The most common diagnosis in the MF/SS group was classic MF (77.3%). Half of the patients with MF had stage IA disease when diagnosed, and the majority were either in partial remission (32.5%) or had stable disease (33.1%). The most widely used treatments were topical corticosteroids (90.8%) and phototherapy. The most common form of primary CBCL was marginal zone lymphoma (50%). Almost all of the patients had cutaneous involvement only and nearly half had stage T1a disease. Most (76.1%) were in complete remission. The main treatments were surgery (55.4%) and radiotherapy (41.9%). The most common diagnosis in patients with CD30 CLPD was lymphomatoid papulosis (68.8%). Most of the patients (31.4%) had stage T3b disease and half were in complete remission. The most common treatments were topical corticosteroids (68.8%) and systemic chemotherapy (32.9%).
The characteristics of patients with primary cutaneous lymphoma in Spain do not differ from those described in other series in the literature. The registry will facilitate clinical research by the AEDV's lymphoma group.
原发性皮肤淋巴瘤并不常见。本文介绍了西班牙皮肤病与性病学会（AEDV）的原发性皮肤淋巴瘤登记处，并报告了第一年的结果。
对原发性皮肤淋巴瘤患者进行疾病登记。参与的医院前瞻性地记录了所有原发性皮肤淋巴瘤患者的诊断、治疗、检查及疾病分期数据。进行了描述性分析。
2017年12月，该登记处包含了来自16所大学医院的639例患者的数据（60%为男性）。按频率排序，最常见的诊断依次为蕈样肉芽肿/塞扎里综合征（MF/SS）（348例，55%）、原发性皮肤B细胞淋巴瘤（CBCL）（184例，29%）、原发性皮肤CD30 T细胞淋巴增殖性疾病（CD30 CLPD）（70例，11%）以及其他类型的T细胞淋巴瘤（37例，5%）。总共记录的病例中有105例（16.5%）为新发病例。MF/SS组最常见的诊断为经典型MF（77.3%）。MF患者中一半在诊断时为IA期疾病，大多数处于部分缓解（32.5%）或病情稳定（33.1%）。最常用的治疗方法是外用糖皮质激素（90.8%）和光疗。原发性CBCL最常见的类型是边缘区淋巴瘤（50%）。几乎所有患者仅累及皮肤，近一半患者为T1a期疾病。大多数（76.1%）处于完全缓解状态。主要治疗方法是手术（55.4%）和放疗（41.9%）。CD30 CLPD患者最常见的诊断是淋巴瘤样丘疹病（68.8%）。大多数患者（31.4%）为T3b期疾病，一半处于完全缓解状态。最常用的治疗方法是外用糖皮质激素（68.8%）和全身化疗（32.9%）。
西班牙原发性皮肤淋巴瘤患者的特征与文献中其他系列报道的特征无异。该登记处将有助于AEDV淋巴瘤小组开展临床研究。
