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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]

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]