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- Title
Diffuse large B-cell lymphoma Richter syndrome in chronic lymphocytic leukemia -- a retrospective analysis of treatment outcomes in Polish Adult Leukemia Study Group.
- Authors
Sośnia, Oktawia; Danecki, Michał; Wąsik-Szczepanek, Ewa; Tryc-Szponder, Jagoda; Iskierka-Jażdżewska, Elżbieta; Majeranowski, Alan; Pruszczyk, Katarzyna; Krzemień, Helena; Bołkun, Łukasz; Paszkiewicz-Kozik, Ewa; Drozd-Sokołowska, Joanna; Kwiatkowski, Jacek; Wiśniewska-Organek, Dominika; Budziszewska, Bożena; Jamroziak, Krzysztof; Hus, Iwona; Lech-Marańda, Ewa; Puła, Bartosz
- Abstract
Introduction: Richter syndrome (RS) is defined as development of an aggressive lymphoma with dismal prognosis in 2-10% of patients suffering from chronic lymphocytic leukemia (CLL). The most often diagnosed variant of RS is diffuse large B-cell lymphoma (DLBCL-RS). The treatment options and risk factors of worse outcome for RS still need to be expanded. Aims: To study the characteristic and treatment outcomes of DLBCL-RS patients. Methods: The retrospective analysis of patients with DLBCL-RS treated in hematology centers between 2011 and 2021. Results: Ninety-nine patients with DLBCL-RS were identified. Median time since CLL diagnosis to Richter transformation was 43.0 months (range 0-252.0). Thirty-seven patients transformed during CLL treatment, 56.7% of them were receiving novel agents (Bruton's tyrosine kinase inhibitor or BCL2 inhibitor). Seventy-six patients had RS treated with intensive chemotherapy protocols, mainly R-CHOP-like regimens. Only 42.3% had response to the treatment (25/76 complete responses and 11/76 partial responses). In forty patients RS was primarily resistant to administered treatment, in 47.5% of these patients p53 pathway aberration was noted. Median overall survival (OS) after transformation was 17.3 months (95% CI 11.9-46.8). Significantly lower OS (3.4 months, 95% CI 14.13 -- not reached) was observed in heavily pretreated CLL patients (>3 lines of therapy preceding transformation). Other factors identified as poor OS predictors in univariate analysis were: ECOG ≥2 (p = 0.0014), hemoglobin level <10 g/dL (p = 0.017) and platelet count <100 × 109/L (p = 0.0065). Median progression-free survival (PFS) was 13.5 months (95% CI 9.6-22.7). Better results were achieved for patients who proceeded to autologous hematopoietic stem cell transplantation (auto-HSCT; n = 8) or allogeneic HSCT (allo-HSCT; n = 10). The median OS in auto-HSCT recipients was 46.1 months, in allo-HSCT -- 18.3 months. Conclusions: Our study represents the largest dataset of DLBCL-RS patients in Polish hematology centers and confirms its poor prognosis. Only proceeding to hematopoietic stem cell transplantation may improve the outcomes.
- Subjects
DIFFUSE large B-cell lymphomas; RICHTER syndrome; CHRONIC lymphocytic leukemia; BRUTON tyrosine kinase; RITUXIMAB; FLUDARABINE; HEMATOPOIETIC stem cell transplantation; TREATMENT effectiveness
- Publication
Acta Haematologica Polonica, 2022, Vol 53, Issue A, p45
- ISSN
0001-5814
- Publication type
Article