Search Results (7)

Background: Burkitt lymphoma is a rare, aggressive B-cell neoplasm with frequent central nervous system (CNS) involvement, treated with intensive multidrug regimens associated with rituximab. The aim of this study was to assess the efficacy, safety, and feasibility of the LMB protocol in adults with BL in a real-world setting. Methods: We included 55 patients with BL diagnosis according to the 2008 WHO classification, treated with LMB protocol associated with rituximab. Low-risk patients (no bone marrow or CNS involvement) were treated in the group B arm, while high-risk patients were placed in group C, which was further stratified by age and CNS infiltration. Results: Thirty-four patients (62%) were treated in group B and 21 patients (38%) were treated in group C, with a median age of 34 years (16–77). Extranodal infiltration was present in 71% patients, including 11 (20%) with CNS involvement. After a median follow up time of 7 years, the complete remission rate was 85%, and progression-free and overall survival at 3 years were 79% and 84%, respectively. Patients with CNS infiltration had an inferior survival rate (55% at 3 years). Grade 3–4 toxicities were frequent, mainly hematologic, infectious, and mucosal. Treatment required substantial supportive care, including 1604 transfusions and 4696 days of hospitalization. Patients over 60 years had poorer outcomes and higher toxicity. Conclusions: The LMB protocol demonstrated high survival rates in adult BL, although at the cost of significant toxicity and considerable health care resource utilization. Outcomes remained suboptimal in patients with CNS involvement despite treatment intensification. Full article

Background/Objectives: Liver transplantation (LT) is often complicated by severe bleeding and coagulopathy. Viscoelastic testing (VET) offers real-time, bedside assessment of coagulation and may improve transfusion management compared to standard tests. This study evaluates the clinical impact of VET implementation during liver transplantation on bleeding, transfusion requirements, complications, and mortality in a single Eastern European tertiary transplant center. Methods: We conducted a single-center before-and-after study comparing patients undergoing LT before and after the implementation of VET. All procedures were performed by the same surgical and anesthetic team using a standardized protocol. Data were collected retrospectively for the Before VET group and prospectively for the After VET group. We compared transfusion requirements, bleeding, complications, and mortality. Results: A total of 59 patients were included, 22 in the After VET group and 37 in the Before VET group. VET implementation was associated with lower intraoperative blood loss (median 4000 mL vs. 6000 mL, p = 0.017) and reduced red blood cell (RBC) transfusion volume (670 mL vs. 1000 mL, p = 0.008). FFP (0.23 vs. 1.59 units, p = 0.007) and platelet use (0.68 vs. 1.81 units, p = 0.035) were also significantly lower in the VET group, while fibrinogen use was higher (3.00 g vs. 2.00 g, p = 0.036). No differences were observed in complication rates or mortality at 30 days and 1 year in this small before-and-after study. Conclusions: VET improved transfusion precision and individualized coagulation management during LT, leading to reduced use of blood products. These findings support the adoption of VET as a standard of care in LT protocols, as it may enhance patient safety, even though no differences in postoperative complications or mortality were observed. Full article

Viscoelastic point-of-care haemostatic resuscitation methods, such as ROTEM or TEG, are crucial in deciding on time-efficient personalised coagulation interventions. International transfusion guidelines emphasise increased patient safety and reduced treatment costs. We analysed care providers’ perceptions of ROTEM to identify perceived strengths and areas for improvement. We conducted a single-centre, mixed qualitative–quantitative study consisting of interviews followed by an online survey. Using a template approach, we first identified themes in the responses given by care providers about ROTEM. Later, the participants rated six statements based on the identified themes on five-point Likert scales in an online questionnaire. Seventy-seven participants were interviewed, and 52 completed the online survey. By analysing user perceptions, we identified ten themes. The most common positive theme was “high accuracy”. The most common negative theme was “need for training”. In the online survey, 94% of participants agreed that monitoring the real-time ROTEM temograms helps to initiate targeted treatment more quickly and 81% agreed that recurrent ROTEM training would be beneficial. Anaesthesia care providers found ROTEM to be accurate and quickly available to support decision-making in dynamic and complex haemostatic situations. However, clinicians identified that interpreting ROTEM is a complex and cognitively demanding task that requires significant training needs. Full article

COVID-19 convalescent plasma (CCP) has been the only specific anti-viral therapy against SARS-CoV-2 available for more than one year. Following the negative results from most randomized controlled trials on its efficacy in COVID-19 hospitalized patients and the availability of anti-spike monoclonal antibodies (mAbs), the use of CCP has subsequently rapidly faded. However, the continuous appearance of new variants of concern (VOCs), most of which escape mAbs and vaccine-elicited neutralizing antibodies (nAbs), has renewed the interest towards CCP, at least in seronegative immunocompetent patients, and in immunocompromised patients not able to mount a protective immune response. We report here the experience of a single Italian hospital in collecting and transfusing CCP in immunocompromised patients hospitalized for severe COVID-19 between October 2021 and March 2022. During this 6-month period, we collected CCP from 32 vaccinated and convalescent regular blood donors, and infused high nAb-titer CCP units (titered against the specific VOC affecting the recipient) to 21 hospitalized patients with severe COVID-19, all of them seronegative at the time of CCP transfusion. Patients’ median age was 66 years (IQR 50–74 years) and approximately half of them (47.6%, 10/21) were immunocompromised. Two patients were rescued after previous failure of mAbs. No adverse reactions following CCP transfusion were recorded. A 28-day mortality rate of 14.3 percent (3/21) was reported, with age, advanced disease stage and late CCP transfusion associated with a worse outcome. This real-life experience also supports the use of CCP in seronegative hospitalized COVID-19 patients during the Delta and Omicron waves. Full article

May–Hegglin anomaly (MHA) is a rare autosomal dominant disorder in the spectrum of myosin heavy chain-related disorders (MYH9-RD), characterized by congenital macrothrombocytopenia and white blood cell inclusions. MHA carries a potential risk of hemorrhagic complications. Bleeding diathesis is usually mild, but sporadic, life-threatening events have been reported. Data regarding the clinical course and outcomes of neonatal MYH9-RD are limited, and specific guidelines on platelet transfusion in asymptomatic patients are lacking. We present monochorionic twins born preterm at 32 weeks of gestation to an MHA mother; both presented with severe thrombocytopenia at birth. Peripheral blood smear demonstrated the presence of macrothrombocytes, and immunofluorescence confirmed the diagnosis of MHA. Close clinical monitoring excluded bleeding complications, and serial hemostatic assessments through a viscoelastic system demonstrated functionally normal primary hemostasis in both patients. Therefore, prophylactic platelet transfusions were avoided. Whole DNA sequencing confirmed the pathogenetic variant of MHA of maternal origin in both twins. Thromboelastography allowed real-time bedside bleeding risk assessment and supported individualized transfusion management in preterm newborns at risk of hemostatic impairment. This report suggests that dynamic and appropriate clotting monitoring may contribute to the more rational use of platelets’ transfusions while preserving patients with hemorrhagic complications and potential transfusion-related side effects. Full article

Beta thalassemia major (βT) is a hereditary anemia characterized by transfusion-dependency, lifelong requirement of chelation, and organ dysfunction. MicroRNA (miRNA) can be packed into extracellular vesicles (EVs) that carry them to target cells. We explored EV-miRNA in βT and their pathophysiologic role. Circulating EVs were isolated from 35 βT-patients and 15 controls. EV miRNA was evaluated by nano-string technology and real-time quantitative polymerase chain reaction (RT-qPCR). We explored effects of EVs on cell culture proliferation, apoptosis, and signal transduction. Higher amounts of small EV (exosomes) were found in patients than in controls. The expression of 21 miRNA was > two-fold higher, and of 17 miRNA < three-fold lower in βT-EVs than control-EVs. RT-qPCR confirmed differential expression of six miRNAs in βT, particularly miR-144-3p, a regulator of erythropoiesis. Exposure of endothelial, liver Huh7, and pancreatic 1.1B4 cells to βT-EVs significantly reduced cell viability and increased cell apoptosis. βT-EV-induced endothelial cell apoptosis involved the MAPK/JNK signal-transduction pathway. In contrast, splenectomized βT-EVs induced proliferation of bone marrow mesenchymal stem cells (BM-MSC). In summary, the miR-144-3p was strongly increased; βT-EVs induced apoptosis and decreased endothelial, pancreatic, and liver cell survival while supporting BM-MSC proliferation. These mechanisms may contribute to βT organ dysfunction and complications. Full article

There is good evidence that 50% or more of red blood cell (RBC) transfusions are unnecessary. To curtail inappropriate RBC transfusions at our hospital, real-time clinical decision support was implemented in our electronic medical record (EMR) that alerts clinicians to the patient’s most recent pretransfusion hemoglobin value upon order entry and provides Best Practice Advisory. This is a soft pop-up alert which is activated when the hemoglobin exceeds 7 g/dL. The ordering clinician can either honor (by cancelling the order) or override the alert. We studied the impact of the alert on blood utilization during a 3-month period (November 2016 to January 2017). For patients who were transfused despite the alert, a retrospective review of the medical chart was performed to determine whether or not the transfusion was clinically indicated. During the study period, 178 of the 895 RBC transfusion orders (20%) triggered the alert. After excluding duplicates, 144 orders were included in our analysis. Most of these orders (124/144, 86%) were carried out despite the alert. According to our chart review, 48% of the alert transfusions could be considered inappropriate, with hemodynamically stable, asymptomatic anemia being the leading indication. Of clinical services, orthopedic surgery had the highest rate of overriding the alert with no clinical justification (70%). The number of RBC transfusions dropped from 313.5 units per month (preintervention period) to 293.2 units per month (postintervention period)—a 6.5% decrease. Real-time clinical decision support may reduce the number of inappropriate RBC transfusions in a community hospital setting, though in our study, the decrease in blood utilization (6.5%) did not reach statistical significance. Full article