Background: Marginal Zone Lymphomas (MZL) include Splenic (SMZL), Nodal (NMZL), and Extranodal (ENMZL) pathologic subtypes and are typically associated with an indolent clinical course. Even if outcomes of most MZL ca...
Background: Marginal Zone Lymphomas (MZL) include Splenic (SMZL), Nodal (NMZL), and Extranodal (ENMZL) pathologic subtypes and are typically associated with an indolent clinical course. Even if outcomes of most MZL cases are excellent, a significant proportion of patients relapse after first line therapy showing heterogenous behavior, with few studies describing outcomes after first relapse. In this report we investigated clinical characteristics and survival of MZL patients prospectively enrolled in the NF10 observational study who experienced a first relapse after induction systemic therapy. Methods: The NF10 Project was started in 2010 by Fondazione Italiana Linfomi (FIL) as a prospective registry specifically conceived to investigate the prognosis of Indolent Non-Follicular B-Cell Lymphomas (INFL). All pts with a histologic confirmed diagnosis of INFL were eligible for NF10 study with no exclusion criteria. In addition to conventional MZL subtypes the category of disseminated MZL (dissMZL) was defined for cases with undefined presentation. For the current study we selected only patients who experienced a first relapse after systemic therapy. Pts were followed and treated based on local institution guidelines. Primary endpoint was Progression free survival from first relapse (PFS2) which was defined as the time from the date of first relapse (Index date) from subsequent progression, re-treatment, or death due to any cause: secondary endpoint was Survival after relapse (SAR) which was calculated from the index date to death. Results: Between July 2010 and July 2018, 740 MZL cases had been registered by 65 centres in Europe and South America. Systemic therapy was administered to 435 patients (pts) at time of diagnosis and to 66 cases after initial watch& wait. Overall, 122 patients were identified who had a first relapse after systemic therapy and represented the study population. At index date 51% were older than 70 years (yrs), 47% were male, 36% 15% 29%, and 20%
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