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This document is an update of the British Society for Haematology (BSH) guideline on myelofibrosis (MF), providing healthcare professionals with clear guidance on the diagnosis and prognostic evaluation of different types of MF. It emphasizes the importance of bone marrow biopsy and molecular testing for diagnosis, as well as the use of various prognostic models to inform clinical decisions and guide therapy. The document also discusses risk stratification and prognostication for MF patients, including the use of validated risk scores. Additionally, it provides information on the diagnosis and prognostication of prefibrotic MF, highlighting the importance of diagnostic criteria and mutational profiles. [Extracted from the article]

Summary: Management of chronic myeloid leukaemia (CML) has recently undergone dramatic changes, prompting the European LeukemiaNet (ELN) to issue recommendations in 2013; however, it remains unclear whether real‐world CML management is consistent with these goals. We report results of UK TARGET CML, a retrospective observational study of 257 patients with chronic‐phase CML who had been prescribed a first‐line TKI between 2013 and 2017, most of whom received first‐line imatinib (n = 203). Although 44% of patients required ≥1 change of TKI, these real‐world data revealed that molecular assessments were frequently missed, 23% of patients with ELN‐defined treatment failure did not switch TKI, and kinase domain mutation analysis was performed in only 49% of patients who switched TKI for resistance. Major molecular response (MMR; BCR‐ABL1IS ≤0·1%) and deep molecular response (DMR; BCR‐ABL1IS ≤0·01%) were observed in 50% and 29%, respectively, of patients treated with first‐line imatinib, and 63% and 54%, respectively, receiving a second‐generation TKI first line. MMR and DMR were also observed in 77% and 44% of evaluable patients with ≥13 months follow‐up, receiving a second‐generation TKI second line. We found little evidence that cardiovascular risk factors were considered during TKI management. These findings highlight key areas for improvement in providing optimal care to patients with CML. [ABSTRACT FROM AUTHOR]

Objectives: We sought to characterise the outcomes of patients with haematological malignancy and severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2) infection in hospital in our regional network of 7 hospitals. Methods: Consecutive hospitalised patients with haematological malignancy and SARS‐CoV‐2 infection were identified from 01/03/2020 to 06/05/2020. Outcomes were categorised as death, resolved or ongoing. The primary outcome was preliminary case fatality rate (pCFR), defined as the number of cases resulting in death as a proportion of all diagnosed cases. Analysis was primarily descriptive. Results: 66 Patients were included, overall pCFR was 51.5%. Patients ≥ 70 years accounted for the majority of hospitalised cases (42, 63%) and fatalities (25, 74%). Mortality was similar between females (52%) and males (51%). Immunosuppressive or cytotoxic treatment within 3 months of the diagnosis of SARS‐CoV‐2 infection was associated with a significantly higher pCFR of 70%, compared with 28% in those not on active treatment (P =.0013, 2 proportions z test). Conclusions: Mortality rates in patients with haematological malignancy and SARS‐CoV‐2 infection in hospital are high supporting measures to minimise the risk of infection in this population. [ABSTRACT FROM AUTHOR]

Twenty-nine patients (97%) received at least two cycles of therapy and one patient discontinued carfilzomib after one cycle. Carfilzomib therapy is ongoing in 17 patients and was discontinued in 13 patients. Five patients received full twice-weekly dosing throughout therapy, and six patients were dose-reduced to weekly upfront (i.e. from cycle 1). Depending on the severity of hypertension, management consisted of introducing an antihypertensive (e.g., amlodipine) in two patients, an antihypertensive dose increase in two patients, carfilzomib dose reduction in two patients, and an antihypertensive plus carfilzomib dose reduction in one patient. [Extracted from the article]

Blast crisis of chronic myeloid leukemia with plasmacytoid dendritic cell phenotype associated with a rare fusion transcript, e13a3 BCR-ABL1 This 30-year-old male patient presented with a pathological fracture of the left distal femur and worsening left shoulder pain (Figure 1(A-C)). Stem cell transplantation can provide durable disease control in blastic plasmacytoid dendritic cell neoplasm: a retrospective study from the European group for blood and marrow transplantation. [Extracted from the article]