Introduction: Treatment of transformed follicular lymphoma (TFL) is diverse as there are no randomized studies to guide therapy. Patients are treated with rituximab (R)-chemotherapy only or with R-chemo and upfront autologous stem cell transplantation (ASCT) at their physicians' discretion. We investigated different treatment modalities and outcome of patients registered in the Population-based Haematological Registry for Observational Studies (PHAROS), covering 40% of the Dutch population. Methods: From the PHAROS registry, we extracted all patients with a diagnosis of both follicular lymphoma (FL) and diffuse large B-cell lymphoma (DLBCL) between January 2004 and July 2013 and checked their histology. Only patients with biopsy-proven DLBCL and underlying FL (previously or simultaneously diagnosed) were included. A separate analysis was performed on patients below 68 years, since upfront ASCT was given up to 67 years. Results: One hundred and sixty-one patients were included, median age at transformation was 63 years (34-91), median time to TFL was 14 months (0-101) and median follow-up after transformation was 17 months (1-111). Former treatment for FL: Sixty-one per cent had been treated with chemotherapy for FL (66% of them with R), 2% had received radiotherapy only, 24% were under a watch-and-wait strategy and, in 13%, FL and DLBCL were diagnosed simultaneously. Treatment for TFL: Ten patients were unable to receive any treatment. All 151 treated patients received R-chemo as induction therapy: 62 patients (33, <68 years) received R-chemo only, and 32 (all <68 years) were in remission after induction Rchemo and received upfront ASCT [23 with R and 9 preceded by 90Y ibritumomab tiuxetan (Z)]. One patient received upfront consolidation with allogeneic SCT. Fiftysix patients (40, <68 years) were primary refractory to induction treatment, of whom 15 (all <68 years) could be rescued by ASCT. When patients had been treated for FL before, significantly more often upfront ASCT was given as treatment of TFL: 33% of untreated patients received upfront ASCT versus 63% of pretreated patients (p < 0.01). In the R-chemo-only group, 39% had been pretreated with chemo and 54% with R, versus 69% and 77% in the upfront ASCT group (p < 0.01). Median OS of all TFL patients was 52 months, with a 2-year OS of 55%. To compare survival between treatment groups, we analysed 109 patients <68 years. After successful R-chemo, 2-year OS was 90%, rising to 96% after upfront ASCT (with or without Z). Two-year OS was 40% after salvage Z-ASCT and 0% when patient was R-chemo refractory and unable to reach salvage ASCT. Conclusion: Although TFL is considered to have a poor prognosis, our data show that when induction R-chemotherapy for TFL is successful, OS is very high. Refractoriness to induction is the main cause of mortality. Physicians offer upfront ASCT more often to patients pretreated with R-chemo for FL. Upfront Z-ASCT seems to result in the highest OS rates, despite more pretreated patients in this group. Studies are needed to identify the patients benefitting most.
M.J., W., D.E., I., J.M., Z., O., V., S., Z., & M.E., C. (2015). First-line treatment and survival of transformed follicular lymphoma in the netherlands in the rituximab era; a population-based analysis. Hematological Oncology, 33, 265–266. Retrieved from http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L72040020 http://dx.doi.org/10.1002/hon.2229