The aim of this study is to assess the incidence, risk factors, and outcome of biopsy-proven transformation in follicular lymphoma (FL) patients in the rituximab era. Transformation was analyzed in 1233 patients with initially diagnosed FL grades 1-3A, identified between 2002 and 2012 in the prospectively maintained Czech Lymphoma Study Group database. Only patients with histologically proven transformation (HT) were included. HT occurred in 58 cases at a median of 3.0 years from the initial FL diagnosis; the HT rate was 4% at 5 years. Transformation occurred most frequently at the first relapse (84% patients). Median OS from the HT was 2.5 years (95% CI 0.4-4.6) and 6-year OS with HT was shorter compared to all FLs (60 vs. 83.9%; 95% CI). A bulky tumor (≥ 10 cm), increased lactate dehydrogenase, age ≥ 60 years, and International Prognostic Index (intermediate/high risk), but not Follicular Lymphoma International Prognostic Index, were associated with transformation (p < 0.05). In the first line, 70% of patients received rituximab (including 36% rituximab maintenance), 57% CHOP-like regimens, and 2.6% of patients were treated with fludarabine-based therapy, whereas 11% of patients were watched only. The patients treated with R-CHOP in the first line (n = 591) showed the transformation rate at 5 years of 4.23% (95% CI 2.52-5.93); subsequent rituximab maintenance (n = 276) vs. observation (n = 153) was associated with a lower transformation rate (p.033; HR 3.29; CI 1.10-9.82). The transformation rate seems to be lower than in previous series, which may be influenced by broad use of rituximab, but prognosis of HT developed during therapy continues to be poor.
- MeSH
- analýza přežití MeSH
- antracykliny škodlivé účinky terapeutické užití MeSH
- biopsie MeSH
- dospělí MeSH
- folikulární lymfom farmakoterapie epidemiologie patologie prevence a kontrola MeSH
- incidence MeSH
- kohortové studie MeSH
- lidé středního věku MeSH
- lidé MeSH
- nádorová transformace buněk účinky léků patologie MeSH
- následné studie MeSH
- protinádorové látky imunologicky aktivní škodlivé účinky terapeutické užití MeSH
- protokoly protinádorové kombinované chemoterapie škodlivé účinky terapeutické užití MeSH
- registrace MeSH
- retrospektivní studie MeSH
- rituximab škodlivé účinky terapeutické užití MeSH
- sekundární prevence MeSH
- senioři MeSH
- udržovací chemoterapie škodlivé účinky MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- Geografické názvy
- Česká republika epidemiologie MeSH
We report a rare case of anaplastic large cell ALK+ lymphoma (ALCL) with initial asymptomatic cardiac involvement. A 59-year-old male with infiltration of the right ventricular wall underwent standard combined chemotherapy (CHOP) and achieved remission without significant cardiac impairment. Additionally, we report the actual incidence of cardiac lymphoma in newly diagnosed non-Hodgkin Lymphomas (NHLs). In total, 16 patients with cardiac lymphoma were found (0.1% NHLs) in the Czech Lymphoma Study Group database. DLBCL was the most frequent histology subtype (50%), and ALCL was identified in 12.5% of cases. At initial diagnosis, the median age was 55.5 (range 21-74) years and 59% were men. None of the 16 patients with cardiac involvement had isolated heart lymphoma. The response to first-line therapy was 79% in 14/16 evaluable patients. The median progression-free survival and overall survival were nearly the same – approximately 3.5 years (range; 0.05-16.7ys), while the median follow up was 4 years.
