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Introduction: Infections represent one of the most frequent causes of death of higher-risk MDS patients, as reported previously also by our group. Azacitidine Infection Risk Model (AIR), based on red blood cell (RBC) transfusion dependency, neutropenia <0.8 x 109/L, platelet count <50 x 109/L, albumin <35g/L, and ECOG performance status ≥2 has been proposed based on the retrospective data to estimate the risk of infection in azacitidine treated patients. Methods: The prospective non-intervention study aimed to identify factors predisposing to infection, validate the AIR score, and assess the impact of antimicrobial prophylaxis on the outcome of azacitidine-treated MDS/AML and CMML patients. Results: We collected data on 307 patients, 57.6 % males, treated with azacitidine: AML (37.8%), MDS (55.0%), and CMML (7.1%). The median age at azacitidine treatment commencement was 71 (range, 18-95) years. 200 (65%) patients were assigned to higher risk AIR group. Antibacterial, antifungal, and antiviral prophylaxis was used in 66.0%, 29.3%, and 25.7% of patients, respectively. In total, 169 infectious episodes (IE) were recorded in 118 (38.4%) patients within the first three azacitidine cycles. In a multivariate analysis ECOG status, RBC transfusion dependency, IPSS-R score, and CRP concentration were statistically significant for infection development (p < 0.05). The occurrence of infection within the first three azacitidine cycles was significantly higher in the higher risk AIR group -- 47.0% than in lower risk 22.4% (odds ratio (OR) 3.06; 95% CI 1.82-5.30, p < 0.05). Administration of antimicrobial prophylaxis did not have a significant impact on all-infection occurrence in multivariate analysis: antibacterial prophylaxis (OR 0.93; 0.41-2.05, p = 0.87), antifungal OR 1.24 (0.54-2.85) (p = 0.59), antiviral OR 1.24 (0.53-2.82) (p = 0.60). Discussion: The AIR Model effectively discriminates infection-risk patients during azacitidine treatment. Antimicrobial prophylaxis does not decrease the infection rate. [ABSTRACT FROM AUTHOR]

Richter transformation (RT) is defined as developing an aggressive lymphoma in 2–10% of patients suffering from chronic lymphocytic leukemia (CLL). So far, no complex analysis of RT demographics and treatment outcomes has been performed in Poland. Thus, the retrospective analysis of 124 patients with RT from Polish hematology centers was designed. Ninety-nine patients with diffuse large B-cell lymphoma (DLBCL-RT) were identified. The median overall survival (OS) for DLBCL-RT was 17.3 months, while for Hodgkin lymphoma (HL-RT)-21.3 months. In multivariate analysis, the independent factors of worse OS for DLBCL-RT were: prior CLL therapy, ECOG stage ≥2, and elevated serum LDH activity. Patients who proceeded to hematopoietic stem cell transplantation (HSCT) achieved better results. The median OS in allogeneic HSCT recipients was not reached, while in autologous HSCT median OS was 51.3 months. In conclusion, our study represents the largest dataset of patients diagnosed with RT in Poland and confirms its dismal prognosis. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]