British Journal of Haematology最新文献

Microvascular, inflammatory and myelin pathologies may contribute to chemotherapy-related cognitive impairment (CRCI). This study applied a novel three-compartment intravoxel incoherent motion-free water imaging (IVIM-FWI) technique that estimates the perfusion fraction (PF), free water fraction (FW) and anisotropic diffusion of tissue (FAt) to study microvascular and microstructural changes in grey and white matter regions in 16 cancer survivor (CS) participants compared to 15 matched healthy controls (HCs). We found significantly decreased PF and increased FW in grey and white matter regions and significantly decreased FAt in white matter regions in the CS versus HC group. These changes were located in key regions involved in emotion, cognition and sensory processing. Furthermore, in both groups, decreased FAt and varying changes in PF and FW were significantly associated with poor performance on cognitive tests assessing general cognitive ability, fluid intelligence, inhibition and processing speed. Overall, the three-compartment IVIM-FWI model provides neuroinflammation, myelination and microvascular metrics that may be related to CRCI pathologies and are associated with cognition. This approach may facilitate targeted interventions aimed at preserving cognitive function and improving overall quality of life for paediatric haematological cancer survivors.

Alemtuzumab in vivo T-cell depletion is safe and effective at a single dose of 30 mg in reduced-intensity matched unrelated donor transplants.

Left panel: Scheme of the XPAG-ITP trial. The dexamethasone monotherapy (DEX) arm consisted of DEX 40 mg/day for days 1-4 for one to three cycles every 28 days to a maximum of 12 weeks, cycles 2 + 3 were optional. Patients randomised to eltrombopag combined with dexamethasone (ETB + DEX) received eltrombopag (ETB) in combination with a short course of high-dose DEX beginning on day 1 (40 mg/day during days 1-4). The starting dose of ETB was 50 mg/day for 2 weeks; thereafter, the ETB dose was increased by 25 mg for all patients who did not achieve the target platelet count of ≥50 × 10⁹/L. The ETB tapering was performed by decreasing the dose by 25 mg every 2 weeks to a minimum dose of 25 mg every other day for all patients. Right upper panel: Clinical outcomes in patients treated with first-line ETB + DEX. Patients displayed a qualitatively longer response duration and qualitatively reduced usage of rescue medication. Right lower panel: The immunological T-cell response was different in treatment responders and non-responders. Patients with sustained response (responders) displayed a high T-cell clonality at baseline. Clones are depicted as bubbles (right side).

Venous thromboembolism (VTE) is a common cause of morbidity and mortality in sickle cell disease (SCD). The aim of the study was to review the safety and effectiveness of primary and secondary anti-coagulation prophylaxis in hospitalized SCD patient population and to identify potential VTE risk factors. Retrospective review of prescribed anti-coagulants in hospitalized SCD patients from 1 January 2015 to 11 July 2023. Pharmacological prophylaxis was provided to those ≥13 years of age. Bleeding and thrombosis outcomes were classified as per the International Society Thrombosis and Haemostasis. Generalized logistic mixed models with a random intercept for each patient were used to assess VTE risk. Seven hundred and ninety hospital encounters among 140 unique patients were reviewed. Median age at admission: 17.2 years (interquartile range [IQR]: 13.9-19.0). Primary and secondary prophylactic anti-coagulation was prescribed in 81.0% (n = 640) and 18.4% (n = 146) hospital encounters respectively. Thirty VTE events were identified among 22 individuals (3.8%; n = 30/790). Seven VTE recurrences/progression (23.3%) occurred. Surgical splenectomy was significantly associated with VTE (53.3%; n = 16/30) versus those without VTE (23.3%; n = 177/760) (odds ratio 4.23, 95% confidence interval [CI] 1.36-13.15; p = 0.0128). Despite the use of pharmacological VTE prophylaxis, 30 VTE events with a thrombosis recurrence/progression rate of 23.3% occurred. Further studies are needed to guide pharmacological prophylaxis for those at greater thrombosis risk.

Central nervous system (CNS) involvement is a known complication of adult T-cell leukaemia/lymphoma (ATL), but its clinical characteristics and incidence still remain unclear. This study characterized the clinical features and incidence of CNS involvement in patients with ATL (CNS-ATL). Overall, 213 patients diagnosed with aggressive ATL were retrospectively analysed at the Kagoshima University Hospital between 2002 and 2021. Of which, 28 (13.1%) developed CNS-ATL. Patients with CNS-ATL observed at ATL diagnosis were classified as acute (n = 21, 75%), lymphoma (n = 1, 3.6%) or chronic (n = 5, 17.9%); CNS-ATL observed post-ATL diagnosis comprised 10.2% (10/98) acute, 1.8% (1/54) lymphoma and 16.1% (5/31) chronic cases. Of the 28 patients, 11 (39.3%) had asymptomatic meningeal involvement detected at prophylactic intrathecal therapy, 10 (35.7%) exhibited neurological symptoms at ATL diagnosis, 4 developed symptomatic CNS involvement during induction therapy and 3 experienced CNS relapse post-treatment. Two-year overall survival of CNS-ATL was significantly lower than that of non-CNS-ATL, whereas 1-year cumulative incidence of CNS involvement post-ATL diagnosis was 12.3%. In conclusion, CNS involvement in ATL tends to occur early in the disease course and is associated with a poor prognosis, with a tendency for a higher incidence in acute-type than in lymphoma-type ATL.