Publications by authors named "Emanuela Marchesini"

A compartmental pharmacokinetics (PK) analysis of new extended half-life FVIII concentrates has never been performed in a large cohort of hemophilia patients. An improved PK analysis of individual outcomes may help to tailor hemophilia replacement treatment. PK outcomes after the infusion of a standard single dose of Efmoroctocog alfa were collected from 173 patients with severe/moderately severe hemophilia A in 11 Italian hemophilia centers.

View Article and Find Full Text PDF

Introduction: Factor IX replacement therapy is used for treatment and prophylaxis of bleeding in haemophilia B. rIX-FP is an extended half-life albumin-fusion protein, which, in clinical studies, has demonstrated prolonged dosing intervals up to 21 days for routine prophylaxis, providing therapeutic benefit.

Aims: To describe dosing frequency and consumption (primary endpoint), efficacy and safety of rIX-FP treatment during routine clinical practice in Italy.

View Article and Find Full Text PDF

Objectives: The present review aims to summarize the state-of-the-art von Willebrand disease (VWD) treatment focusing on specific clinical settings (obstetrics, surgery, long-term prophylaxis and comorbidities) as well as on the use of a Von Willebrand factor (VWF) concentrate with low FVIII content.

Methods: Literature research and case reports.

Results And Conclusions: Considering that patients affected by VWD have an intact ability to synthesize FVIII, in order to avoid excessive levels of FVIII, a highly purified plasma VWF concentrate with low FVIII content could be particularly useful in those patients and clinical circumstances at high thrombotic risk as well as for long-term prophylaxis.

View Article and Find Full Text PDF

Background And Aim: Risk factors and mortality in patients with DOACs-associated gastrointestinal bleeding (GIB) are not completely defined. Aims of this study were to identify risk factors for bleeding and evaluate one-year mortality in patients with DOACs-associated GIB.

Methods: We conducted a case-control study.

View Article and Find Full Text PDF

Progress in hemophilia therapy has been remarkable in the first 20 years of the third millennium, but the innovation began with the description the fractionation of plasma in 1946. The first concentrates followed the discovery of FVIII in the cryoprecipitate of frozen plasma and FIX in the supernatant in the early 1960s, which led to the initial attempts at replacement therapy. Unfortunately, the lack of screening methods for viral pathogens resulted in people with hemophilia (PWH) receiving concentrates contaminated by hepatitis A virus, hepatitis C virus, and human immunodeficiency virus, as these concentrates were made from large industrial pools of plasma derived from thousands of donors.

View Article and Find Full Text PDF

Introduction: A number of new FVIII/IX concentrates enriched the portfolio of products available for the treatment of hemophilia A/B patients. Due to the large inter-patient variability, accurate tailoring of the therapy became essential to improve patients' adherence, clinical outcomes, and cost/effectiveness ratio. Recently, non-replacement therapies have taken the limelight and succeeded in decreasing the bleedings of patients.

View Article and Find Full Text PDF

Emicizumab has been approved in several countries for regular prophylaxis in patients with congenital haemophilia A and FVIII inhibitors because it substantially reduces their bleeding risk and improves quality of life. However, although significantly less frequent, some breakthrough bleeds may still occur while on emicizumab, requiring treatment with bypassing or other haemostatic agents. Thrombotic complications have been reported with the associated use of activated prothrombin complex concentrates.

View Article and Find Full Text PDF

Background: In older people, multiple chronic ailments lead to the intake of multiple medications (polypharmacy) that carry a number of negative consequences (adverse events, prescription and intake errors, poor adherence, higher mortality). Because ageing patients with haemophilia (PWHs) may be particularly at risk due to their pre-existing multiple comorbidities (arthropathy, liver disease), we chose to analyse the pattern of chronic drug intake in a cohort of PWHs aged 60 years or more.

Patients And Methods: S + PHERA is a multicentre observational study, with the broad goal to evaluate prospectively the health status and medication intake in 102 older patients with severe haemophilia A or B compared with 204 age- and residence-matched controls chosen randomly from the same general practices of PWHs.

View Article and Find Full Text PDF

Background: Limited data are available on the use of direct oral anticoagulants (DOACs) in patients with cancer and atrial fibrillation (AF).

Methods: Consecutive patients with non-valvular AF treated with DOACs were enrolled in a prospective cohort with the aim of evaluating thromboembolic (ischemic stroke or transient ischemic attack or systemic embolism) and major bleeding (MB) events according to presence and type of cancer. The risk of study outcomes over time was compared using Kaplan-Meier method and log-rank test or Cox proportional hazards regression.

View Article and Find Full Text PDF
Article Synopsis
  • * Challenges still exist in accurately diagnosing vWD and tailoring treatment strategies for individual patients, which can affect therapy effectiveness.
  • * A meeting among Italian haematologists aimed to address these issues by discussing diagnostics, identifying patient subgroups for treatment, and optimizing pre- and post-operative care for vWD patients.
View Article and Find Full Text PDF

Background: Current guidelines recommend vitamin K antagonists (VKAs) or non-vitamin K antagonist oral anticoagulants (NOACs) for stroke prevention in patients with non-valvular atrial fibrillation (AF).

Methods: We compared the clinical features of consecutive in- and out-patients with non-valvular AF newly-treated with NOACs or on treatment with VKAs.

Results: Overall, 1314 patients newly-treated with NOACs and 1024 on treatment with VKAs were included in the study.

View Article and Find Full Text PDF

The development of inhibitory antibodies to factor VIII (FVIII) is a major obstacle in using this clotting factor to treat individuals with hemophilia A. Patients with a congenital absence of FVIII do not develop central tolerance to FVIII, and therefore, any control of their FVIII-reactive lymphocytes relies upon peripheral tolerance mechanisms. Indoleamine 2,3-dioxygenase 1 (IDO1) is a key regulatory enzyme that supports Treg function and peripheral tolerance in adult life.

View Article and Find Full Text PDF

Background: The hallmark of severe hemophilia is recurrent bleeding into joints and soft tissues with progressive joint damage, notwithstanding on-demand treatment. Prophylaxis has long been used but not universally adopted because of medical, psychosocial, and cost controversies.

Objectives: To determine the effectiveness of clotting factor concentrate prophylaxis in the management of people with hemophilia A or B.

View Article and Find Full Text PDF

Background: Antiviral treatment for chronic hepatitis C may be less effective if patients are co-infected with human immunodeficiency virus (HIV).

Objectives: To assess the benefits and harms of antiviral treatment for chronic hepatitis C in patients with HIV.

Search Strategy: Trials were identified through manual and electronic searches in The Cochrane Hepato-Biliary Group Controlled Trials Register, the Cochrane Central Register of Controlled Trials (CENTRAL) in The Cochrane Library, MEDLINE, EMBASE, and Science Citation Index Expanded.

View Article and Find Full Text PDF

Objectives: The aim of this study was to assess the effects of peginterferon plus ribavirin for chronic hepatitis C in patients with human immunodeficiency virus (HIV).

Methods: Trials were identified through manual and electronic searches. Randomized trials comparing peginterferon plus ribavirin with other antiviral treatments for patients with chronic hepatitis C and HIV were included.

View Article and Find Full Text PDF

Background: Fresh-frozen plasma (FFP) is unanimously recognised by international guidelines as the blood component of choice for the management of acute haemorrhage when accompanied by disorders of haemostasis, for disseminated intravascular coagulation in the presence of haemorrhage, for rare bleeding disorders when specific clotting factor concentrates are not available and for thrombotic thrombocytopenic purpura. The literature, however, reports a high percentage of inappropriate requests for FFP. This article presents the results of a pilot study of clinical auditing of the use of FFP in the Region of Umbria (Italy).

View Article and Find Full Text PDF

Background: The clinical use of fresh-frozen plasma (FFP) is progressively increasing both nationally and internationally, despite the fact that many studies have shown the weaknesses of the indications for its use. Guidelines on the good use of plasma have, therefore, been adopted in various countries. The aim of the present study was to analyse some of the existing guidelines on the good use of plasma, applying a scientifically validated method, as a preliminary step in the implementation of Regional guidelines.

View Article and Find Full Text PDF