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Hairy cell leukemia (HCL) and HCL‐like disorders have to be distinguished because of their different biology and treatment response. Thus, we conducted a retrospective study on patients with HCL and hairy cell leukemia variant (HCLv) to assess diagnostic algorithms and treatment outcomes in a real‐world setting. We analyzed 225 HCL and 26 HCLv patients with median follow‐up of 67.9 months (HCL) and 20.1 months (HCLv). Median age at diagnosis was 56.2 (HCL) and 69.5 years (HCLv), male predominance was observed in both groups (76.0% vs. 73.1%). Diagnostics was mostly based on morphological evidence of hairy cells in the peripheral blood and bone marrow. At diagnosis, BRAFV600E mutation was detected in 94.7% of examined HCL patients and in no HCLv patient. Front‐line treatment was indicated in 205 (91.1%) HCL and 18 (69.2%) HCLv patients. The majority of HCL patients were administered a cladribine‐based regimen (91.2%). Overall response rate (ORR) was higher in cladribine‐treated patients compared to those given other treatments (97.7% vs. 81.3%), the same applied with achieving Complete remission (CR) (91.2% vs. 62.5%). HCLv treatment was heterogeneous, but cladribine remained the most frequent option (44.4%) with ORR 81.3% and CR rates 43.8%. Second‐line treatment was indicated in 52 HCL and 8 HCLv patients, 25.4% and 44.4% of those treated in first‐line. In the whole HCL group, median time to next treatment (TTNT) was not reached and 10‐year TTNT was estimated at 74.1%. HCLv patients who underwent first‐line treatment had a median TTNT of 56 months. The median overall survival (OS) in HCL patients was not reached compared to HCLv with a median OS of 9.5 years. These data confirm an excellent prognosis for HCL patients treated with cladribine‐based therapy. On the contrary, HCLv with its aggressive behavior represents a group of patients in whom novel treatment approaches are needed. [ABSTRACT FROM AUTHOR]

Summary: Idelalisib (idela), a phosphatidylinositol 3‐kinase inhibitor, and ibrutinib, a Bruton tyrosine kinase inhibitor, were the first oral targeted agents approved for relapsed/refractory (R/R) chronic lymphocytic leukaemia (CLL). However, no randomised trials of idelalisib plus rituximab (R‐idela) versus ibrutinib have been conducted. Therefore, we performed a real‐world retrospective analysis of patients with R/R CLL treated with R‐idela (n = 171) or ibrutinib (n = 244). The median age was 70 versus 69 years, with a median of two previous lines. There was a trend towards higher tumour protein p53 (TP53) aberrations and complex karyotype in the R‐idela group (53% vs. 44%, p = 0.093; 57% vs. 46%, p = 0.083). The median progression‐free survival (PFS) was significantly longer with ibrutinib (40.5 vs. 22.0 months; p < 0.001); similarly to overall survival (OS; median 54.4 vs. 37.7 months, p = 0.04). In multivariate analysis, only PFS but not OS remained significantly different between the two agents. The most common reasons for treatment discontinuation included toxicity (R‐idela, 39.8%; ibrutinib, 22.5%) and CLL progression (27.5% vs. 11.1%). In conclusion, our data show significantly better efficacy and tolerability of ibrutinib over R‐idela in patients with R/R CLL treated in routine practice. The R‐idela regimen may still be considered a reasonable option in highly selected patients without a suitable treatment alternative. [ABSTRACT FROM AUTHOR]

Until recently, a combination of anti-CD20 antibody plus less intensive chemotherapy was a standard of care in elderly population with previously untreated chronic lymphocytic leukemia (CLL). The aim of this observational study was to retrospectively assess efficacy and safety of obinutuzumab + chlorambucil (G-Clb), rituximab + chlorambucil (R-Clb), and bendamustine + rituximab (BR) given as the frontline therapy within routine practice. The final analyzed dataset included 398 consecutive CLL patients from 10 hematology centers cooperating within the Czech CLL Study Group: 63 treated with G-Clb, 78 with R-Clb, and 257 with BR. There were no significant differences in prognostic and predictive markers among the groups. On the contrary, median age at the start of therapy and cumulative illness rating scale (CIRS) score was significantly higher in R-Clb group. Obinutuzumab plus chlorambucil regimen was preferably offered to elderly patients (compared to BR) with less severe comorbidities and lower CIRS score (compared to R-Clb). A time period when a treatment was indicated had also a strong impact on the choice of the regimen. The overall response rate reached 76% (30% complete remissions, CRs) in G-Clb, 75% (22% CRs) in R-Clb, and 85% (47% CRs) in BR group. Median event-free survival was 49.0 months for G-Clb, 20.3 months for R-Clb, and 37.0 months for BR group. Neutropenia grade ≥ 3 developed in 43% of G-Clb, 31% of R-Clb and in 49% of BR patients, grade ≥ 3 infections were recorded in 17% of G-Clb, 6.4% of R-Clb, and 17% of BR patients. In conclusion, real-world therapeutic activity of G-Clb appears to be at least comparable to prospective clinical trial data. R-Clb yields relatively good results in very old and severely comorbid patients. [ABSTRACT FROM AUTHOR]

Background There are minimal data about the efficacy of subsequent therapy in patients with relapse after FCR (fludarabine, cyclophosphamide, and rituximab) chemoimmunotherapy. Methods We retrospectively analyzed the outcomes of 119 patients who relapsed after standard-dose FCR. The patient cohort consisted of patients who relapsed after FCR administered as first-line therapy ( Group 1, n = 63) and patients relapsing after FCR administered in second/subsequent line; ( Group 2, n = 56). Results Basic parameters (age, clinical stage, cytogenetics, molecular genetics) did not differ significantly between these subgroups. Likewise, median progression-free survival ( PFS) was not considerably different after FCR (18.6 vs. 14.7 months). Subsequent therapy for relapsed disease included FCR retreatment, R- CHOP, alemtuzumab, or rituximab plus high-dose dexamethasone. Overall response rates for the two groups did not significantly differ (59% vs. 44%). Although PFS after subsequent therapy was relatively short, longer PFS was observed in Group 1 (13.3 vs. 5.9 months; P = 0.01), in patients with response duration ≥24 months after previous FCR (13 vs. 6.1 months; P < 0.01), and in patients who achieved complete remission after FCR (10.8 vs. 7.9 months in partial remission; P = 0.01). Newly detected 17p deletions were observed in 5/62 patients, and new p53 mutations in 6/34 FCR-treated patients. Conclusion Our data indicate that the prognosis of patients who relapse after FCR remains poor regardless of the subsequent treatment regimen. [ABSTRACT FROM AUTHOR]