Bleeding pattern and consumption of factor VIII concentrate in adult patients with haemophilia A without inhibitors in the Czech Republic between 2013 and 2021 (Czech National Haemophilia Programme registry data).

The manuscript provides an overview of treatment and its changes in adult patients with haemophilia A without inhibitors in the Czech Republic between 2013 and 2021 using data from the registry of the Czech National Haemophilia Programme (CNHP). Over a 9-year period, we focused on the reduction in the annual bleeding rate (ABR), joint bleeding rate (AJBR) and factor VIII consumption when patients with severe haemophilia A switched from on-demand treatment to prophylaxis. The ABR and AJBR include both patient-reported home treatment and treated hospitalisation episodes. All adult patients with severe haemophilia A were categorised into three groups according to the therapeutic regimen. The first group was patients on prophylaxis during the follow-up period, the second group consisted of patients on on-demand treatment, and the third group was patients who received both treatment regimens during follow-up. With an increase in the proportion of patients with severe haemophilia A on prophylaxis from 37 to 74% between 2013 and 2021, the ABR for all patients with severe haemophilia A decreased approximately 6.9-fold, and the AJBR decreased 8.7-fold. Expectedly, the factor consumption increased by approximately 68.5%. In the group of patients with severe haemophilia A who had switched from an on-demand to a prophylactic regimen, the total number of bleeding events decreased 3.5-fold, and the number of joint bleeding episodes decreased 3.9-fold. Factor VIII consumption increased by 78.4%. Our study supports a previously reported positive effect of prophylaxis on bleeding control. We believe that the substantial improvement in ABR justifies the increased treatment costs.

[The first experience with dabigatran antidote in the University Hospital Pilsen]. / První zkusenosti s podáním antidota dabigatran etexilátu ve FN Plzen.

Dabigatran (Pradaxa, Boehringer Ingelheim) is the first direct oral thrombin (FIIa) inhibitor. It is indicated for thromboprophylaxis in patients undergoing elective total replacement hip or knee joint surgery or for the primary or secondary prophylaxis of elderly patients with non-valvular atrial fibrillation or for deep vein thrombosis and pulmonary embolism treatment and prophylaxis. Idarucizumab (Praxbind, Boehringer Ingelheim) is a specific monoclonal antibody fragment indicated in patients treated with dabigatran. It is recommended when reversal of anticoagulant effect of dabigatran is needed especially in cases of life-threatening bleeding, for emergency surgery or urgent procedures purposes. In this article, there is a summary of our clinical and laboratory experience with reversal effect of idarucizumab in our five patients.

Understanding the value of monocyte distribution width (MDW) in acutely ill medical patients presenting to the emergency department: a prospective single center evaluation.

The monocyte distribution width (MDW) has emerged as a promising biomarker for accurate and early identification of patients with potentially life-threatening infections. Here we tested the diagnostic performance of MDW in adult patients requiring hospital admission for community-acquired infections and sepsis, evaluated sources of heterogeneity in the estimates of diagnostic accuracy, and assessed the meaning of MDW in a patient population presenting to the emergency department (ED) for acute non-infectious conditions. 1925 consecutive patients were categorized into three groups: non-infection (n = 1507), infection (n = 316), and sepsis/septic shock (n = 102). Diagnostic performance for infection or sepsis of MDW alone or in combination with components of SOFA was tested using AUC of ROC curves, sensitivity, and specificity. The relationship between MDW and different pathogens as well as the impact of non-infectious conditions on MDW values were explored. For the prediction of infection, the AUC/ROC of MDW (0.84) was nearly overlapping that of procalcitonin (0.83), and C-reactive protein (0.89). Statistical optimal cut-off value for MDW was 21 for predicting infection (sensitivity 73%, specificity 82%) and 22 for predicting sepsis (sensitivity 79%, specificity 83%). The best threshold to rule out infection was MDW ≤ 17 (NPV 96.9, 95% CI 88.3-100.0), and ≤ 18 (NPV 99.5, 95% CI 98.3-100.0) to rule out sepsis. The combination of MDW with markers of organ dysfunction (creatinine, bilirubin, platelets) substantially improved the AUC (0.96 (95% CI 0.94-0.97); specificity and sensitivity of 88% and 94%, respectively). In conclusion, MDW has a good diagnostic performance in diagnosing infection and sepsis in patients presenting in ED. Its use as an infection marker even increases when combined with other markers of organ dysfunction. Understanding the impact of interactions of non-infectious conditions and comorbidities on MDW and its diagnostic accuracy requires further elucidation.

Prevalence of cancer in patients with superficial vein thrombosis and its clinical importance.

OBJECTIVE: The objective of the present study was to evaluate the prevalence of cancer in patients with superficial vein thrombosis (SVT) of the legs. Moreover, we evaluated the potential determinants of SVT complications by comparing a subgroup with isolated SVT and a subgroup of SVT complicated by concurrent deep vein thrombosis (DVT) and/or pulmonary embolism (PE) with respect to the presence of cancer and other clinical and laboratory characteristics. METHODS: The present single-center, retrospective study of prospectively collected data was conducted in a tertiary care setting. We included patients who had been treated in the thrombosis clinic from 2006 to 2018 for symptomatic SVT of the legs, either isolated SVT or SVT complicated by concurrent DVT/PE. We evaluated the prevalence and type of malignancy (diagnosed ≤12 months before SVT and/or ongoing therapy), demographics, and clinical and laboratory characteristics of the patients. For statistical evaluation, we used the Student t test, Kruskal-Wallis test, Fisher exact two-sided test, and logistic regression. RESULTS: Of 276 patients with SVT (mean age, 58.9 ± 14.7 years; 60.9% women), 191 had had isolated SVT and 85 had had SVT complicated by concurrent DVT/PE. The prevalence of malignancy was 8.7% in the whole group (mainly breast and urinary tract cancer), including 4.2% of those with isolated SVT and 18.8% of those with SVT and concurrent DVT/PE (P < .001). Between the two subgroups, no significant differences were present in the duration of leg symptoms, family or personal history of SVT and/or DVT, SVT location, and smoking. In logistic regression, several factors were significantly associated with the concurrent presence of DVT/PE: age (odds ratio [OR], 1.024; 95% confidence interval [CI], 1.004-1.044), female gender (OR, 0.545; 95% CI, 0.309-0.960), varicose vein SVT (OR, 0.42; 95% CI, 0.194-0.902), thrombophilia (OR, 1.939; 95% CI, 1.089-3.454), and cancer (OR, 4.727; 95% CI, 1.814-12.316). CONCLUSIONS: The prevalence of malignancy in the patients with SVT was 8.7%. Age, thrombophilia, male gender, nonvaricose vein SVT, and cancer were significantly associated with the presence of concurrent DVT/PE. Cancer was the strongest determinant of concurrent DVT/PE.

Repeated Administration of Idarucizumab to a Patient with Dabigatran Overdose.

Idarucizumab is designed to reverse the anticoagulant effect of dabigatran. This case report describes the administration of three independent doses of idarucizumab to a 76-year-old man suffering from atrial flutter being treated with dabigatran to prevent ischaemic stroke. The last dose of dabigatran was administered in the morning of the same day the patient was transferred to hospital because of the need for urgent pericardium puncture. Baseline dTT (dilute thrombin time) reached 700 ng/mL as glomerular filtration (GF) dropped to 0.19 mL/s. The first dose of idarucizumab was administered prior to puncture and the clinical state was stabilized for the next 24 hours although dTT climbed to 400 ng/mL. The need for cannulation before dialysis required a second dose of antidote and a third dose was given prior to removal of the pericardium drain. Monitoring of dabigatran recovery showed that the reverse effect of idarucizumab lasted 12 hours, which is sufficiently long and reliable for urgent situations. Idarucizumab administration is not limited by age and/or renal impairment. We recommend repeated dTT examinations when the dabigatran baseline level exceeds 200 ng/mL and the antidote has been administered.