Morello Lucia, Rattotti Sara, Giordano Laura, Jerkeman Mats, van Meerten Tom, Krawczyk Katarzyna, Moita Filipa, Marino Dario, Ferrero Simone, Szymczyk Michał, Aurer Igor, El-Galaly Tarec Christoffer, Di Rocco Alice, Visco Carlo, Carli Giuseppe, Defrancesco Irene, Carlo-Stella Carmelo, Dreyling Martin, Santoro Armando, Arcaini Luca
Department of Oncology and Hematology, Humanitas Clinical and Research Center, Rozzano, Italy.
Division of Hematology, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy.
Hemasphere. 2019 Dec 16;4(1):e302. doi: 10.1097/HS9.0000000000000302. eCollection 2020 Feb.
While classical nodal mantle cell lymphoma (cMCL) is often associated with involvement of multiple extranodal sites, isolated extranodal disease (ED) at the time of diagnosis is a rare event; data on the outcome of these forms are lacking. On behalf of the European MCL Network, we conducted a retrospective analysis on the clinical characteristics and outcomes of MCL presenting with isolated or predominant ED (MALT MCL). We collected data on 127 patients with MALT MCL diagnosed from 1998 to 2015: 78 patients (61%) were male with a median age of 65 years. The involved sites include: upper airways + Waldeyer ring (40; 32%), gastrointestinal tract (32; 25%), ocular adnexa (17; 13%), oral cavity and salivary glands (17; 13%) and others (13; 1%); 7 patients showed multiple extranodal sites. The median follow-up was 80 months (range: 6-182), 5-year progression-free survival (PFS) was 45% (95% CI: 35-54) and 5-year overall survival (OS) was 71% (95% CI: 62-79). In an explorative setting, we compared MALT MCL with a group of 128 cMCL patients: MALT MCL patients showed a significantly longer PFS and OS compared with nodal cMCL; with a median PFS of 4.5 years vs 2.8 years (p = 0.001) and median OS of 9.8 years vs 6.9 years (p = 0.018), respectively. Patients with MALT MCL at diagnosis showed a more favorable prognosis and indolent course than classical nodal type. This clinical variant of MCL should be acknowledged to avoid possible over-treatment.
虽然经典型结内套细胞淋巴瘤（cMCL）常与多个结外部位受累相关，但诊断时孤立性结外病变（ED）是罕见情况；目前缺乏关于这些类型预后的数据。代表欧洲MCL网络，我们对表现为孤立性或主要为结外病变的套细胞淋巴瘤（MALT MCL）的临床特征和预后进行了回顾性分析。我们收集了1998年至2015年诊断的127例MALT MCL患者的数据：78例（61%）为男性，中位年龄65岁。受累部位包括：上呼吸道+瓦尔代尔环（40例；32%）、胃肠道（32例；25%）、眼附属器（17例；13%）、口腔和唾液腺（17例；13%）及其他（13例；1%）；7例患者表现为多个结外部位。中位随访时间为80个月（范围：6 - 182个月），5年无进展生存期（PFS）为45%（95%置信区间：35 - 54），5年总生存期（OS）为71%（95%置信区间：62 - 79）。在一项探索性研究中，我们将MALT MCL与一组128例cMCL患者进行了比较：MALT MCL患者的PFS和OS显著长于结内cMCL；中位PFS分别为4.5年和2.8年（p = 0.001），中位OS分别为9.8年和6.9年（p = 0.018）。诊断时患有MALT MCL的患者比经典结内型预后更良好，病程更惰性。应认识到MCL的这种临床变异型，以避免可能的过度治疗。
