Ortega José L, Cabanillas Fernando, Rivera Noridza, Tirado-Gomez Maribel, Hallman Deana, Pardo Wandaly I, Bruno Margarita
University District Hospital, University of Puerto Rico School of Medicine, San Juan, Puerto Rico.
University District Hospital, University of Puerto Rico School of Medicine, San Juan, Puerto Rico; Hospital Auxilio Mutuo Cancer Center, San Juan, Puerto Rico.
Clin Lymphoma Myeloma Leuk. 2017 Dec;17(12):879-883. doi: 10.1016/j.clml.2017.09.014. Epub 2017 Sep 23.
Marginal zone lymphomas (MZLs) are indolent disorders composed of 3 subtypes: extranodal marginal zone lymphoma (MALT), splenic marginal zone lymphoma (SMZL), and nodal marginal zone lymphoma (NMZL). Early-stage MALT is treated with radiotherapy or antibiotics, and advanced MALT and NMZL are managed with either watch and wait or chemotherapy. SMZLs are treated with splenectomy or rituximab. However, because these approaches have failed to cure patients with SMZL and NMZL, we have systematically used upfront chemotherapy for them, as well as for advanced MALT. We report the outcomes of this approach.
A total of 44 patients with MZL were identified from our database and divided into 2 groups. Group 1 (22 with early-stage MALT) patients received either radiotherapy (n = 17) or antibiotics with or without surgery (n = 5). Group 2 included 9 patients with advanced MALT, 9 with SMZL, and 4 with NMZL. Group 2 was treated with FND-R (fludarabine 25 mg/m on days 1 to 3, mitoxantrone 10 mg/m on day 1, dexamethasone 20 mg on days 1 to 5, and rituximab 375 mg/m on day 1; n = 14) or CHOP-R (cyclophosphamide 750 mg/m on day 1, doxorubicin 50 mg/m on day 1, vincristine 2 mg intravenous push on day 1, prednisone 100 mg/m orally on days 1 to 5, rituximab 375 mg/m on day 1; n = 8), followed by maintenance rituximab for 70%.
All patients achieved complete remission, and only 2 patients in group 1 had developed a relapse at 70 and 75 months. Both relapses were stage I MALT that had initially been treated with radiotherapy. Both were salvaged with FND-R and remained free of disease at 27 and 39 months after the relapse. At 10 years, the failure-free survival for the 44 patients was 80% and the overall survival was 100%. None of the patients in group 2 developed a relapse. The long-term toxicities have been acceptable.
The excellent responses using upfront chemotherapy for MZL suggests that this disorder is curable. Our results should be confirmed in a prospective trial.
边缘区淋巴瘤（MZL）是由3种亚型组成的惰性疾病：结外边缘区淋巴瘤（MALT）、脾边缘区淋巴瘤（SMZL）和淋巴结边缘区淋巴瘤（NMZL）。早期MALT采用放疗或抗生素治疗，晚期MALT和NMZL采用观察等待或化疗。SMZL采用脾切除术或利妥昔单抗治疗。然而，由于这些方法未能治愈SMZL和NMZL患者，我们对它们以及晚期MALT系统性地采用了初始化疗。我们报告了这种方法的结果。
从我们的数据库中确定了44例MZL患者，并分为2组。第1组（22例早期MALT患者）接受放疗（n = 17）或抗生素治疗（有或无手术，n = 5）。第2组包括9例晚期MALT患者、9例SMZL患者和4例NMZL患者。第2组接受FND-R方案（氟达拉滨25 mg/m²，第1至3天；米托蒽醌10 mg/m²，第1天；地塞米松20 mg，第1至5天；利妥昔单抗375 mg/m²，第1天；n = 14）或CHOP-R方案（环磷酰胺750 mg/m²，第1天；多柔比星50 mg/m²，第1天；长春新碱2 mg静脉推注，第1天；泼尼松100 mg/m²，口服，第1至5天；利妥昔单抗375 mg/m²，第1天；n = 8），随后70%的患者接受利妥昔单抗维持治疗。
所有患者均达到完全缓解，第1组仅2例患者在70和75个月时复发。两次复发均为最初接受放疗的I期MALT。两者均采用FND-R方案挽救，复发后27和39个月时仍无疾病。10年时，44例患者的无失败生存率为80%，总生存率为100%。第2组无患者复发。长期毒性反应可接受。
对MZL采用初始化疗取得的优异反应表明这种疾病是可治愈的。我们的结果应在前瞻性试验中得到证实。
