Augusto B. Federici
YOU?
Author Swipe
View article: The landscape of rare coagulation factor deficiency management in Italy: a national hemophilia center survey
The landscape of rare coagulation factor deficiency management in Italy: a national hemophilia center survey Open
Background: Rare bleeding disorders (RBDs) are a group of inherited conditions caused by deficiencies in specific coagulation factors, excluding hemophilia A/B and von Willebrand disease. Individually rare, they pose a significant challeng…
View article: Clinical, laboratory, and molecular markers of type 3 von Willebrand disease
Clinical, laboratory, and molecular markers of type 3 von Willebrand disease Open
Type 3 von Willebrand disease (VWD3) is due to a virtually complete deficiency of von Willebrand factor (VWF), and it is inherited as an autosomal recessive trait: heterozygous relatives have mild or no bleeding symptoms. The prevalence of…
View article: Clinical and molecular markers of VWD2B
Clinical and molecular markers of VWD2B Open
Type 2B von Willebrand disease (VWD2B) is a rare inherited bleeding disorder caused by VWF mutants with heightened binding to the platelet receptor glycoprotein Ib alpha (GpIbα), as demonstrated by enhanced ristocetin-induced platelet aggr…
View article: Real-World Efficacy and Safety of Plasma-Derived Von Willebrand Factor-Containing Factor VIII Concentrates in Patients With Von Willebrand Disease in Italy
Real-World Efficacy and Safety of Plasma-Derived Von Willebrand Factor-Containing Factor VIII Concentrates in Patients With Von Willebrand Disease in Italy Open
Plasma-derived von Willebrand factor-containing factor VIII concentrates (pd-VWF/FVIII-C) are the mainstay of treatment in von Willebrand disease (VWD). Real-world data on efficacy and safety of these pd-VWF/FVIII-C are required. To retros…
View article: Von Willebrand Disease: Gaining a global perspective
Von Willebrand Disease: Gaining a global perspective Open
Introduction Recent guidelines for von Willebrand Disease (VWD) highlighted the challenges in diagnosis and management. Identifying the number of persons with VWD (PwVWD) internationally will help target support to aid diagnosis of PwVWD. …
View article: Lung clearance index short-term variability in cystic fibrosis. A pre-post pulmonary exacerbation study.
Lung clearance index short-term variability in cystic fibrosis. A pre-post pulmonary exacerbation study. Open
Background: Multiple Breath washout (MBW) represents an important tool to detect early a possible pulmonary exacerbation especially in Cystic Fibrosis (CF) disease. Lung clearance index (LCI) is the most commonly reported multiple breath w…
View article: WEB-REHAB Program for people with Cystic Fibrosis during Covid-19 pandemic: a feasibility study
WEB-REHAB Program for people with Cystic Fibrosis during Covid-19 pandemic: a feasibility study Open
Introduction: Telemedicine and tele-monitoring represent an emerging-study area in several and chronic diseases. Tele-rehabilitation during Covid-19 disease became an essential tool to promote physical activity in total safety. Infact, for…
View article: SARS‐CoV‐2 infection in patients with chronic lymphocytic leukemia: The Italian Hematology Alliance on COVID‐19 cohort
SARS‐CoV‐2 infection in patients with chronic lymphocytic leukemia: The Italian Hematology Alliance on COVID‐19 cohort Open
COVID‐19, the disease caused by SARS‐CoV‐2, is still afflicting thousands of people across the globe. Few studies on COVID‐19 in chronic lymphocytic leukemia (CLL) are available. Here, we analyzed data from the CLL cohort of the Italian He…
View article: A need to increase von Willebrand disease awareness: vwdtest.com – A global initiative to help address this gap
A need to increase von Willebrand disease awareness: vwdtest.com – A global initiative to help address this gap Open
Von Willebrand disease (VWD) is an inherited bleeding disorder caused by quantitative or qualitative deficiencies in von Willebrand factor (VWF). People with VWD may experience excessive, recurrent or prolonged bleeding, particularly durin…
View article: Sixth Åland Island Conference on von Willebrand disease
Sixth Åland Island Conference on von Willebrand disease Open
Introduction The sixth Åland Islands Conference on von Willebrand disease (VWD) on the Åland Islands, Finland, was held from 20 to 22 September 2018. Aim The meeting brought together experts in the field of VWD from around the world to sha…
View article: Use of the von Willebrand factor concentrate with low factor VIII content to manage patients with inherited von Willebrand disease requiring surgical or secondary long‐term prophylaxis: An expert opinion paper from an Italian panel
Use of the von Willebrand factor concentrate with low factor VIII content to manage patients with inherited von Willebrand disease requiring surgical or secondary long‐term prophylaxis: An expert opinion paper from an Italian panel Open
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 Vo…
View article: Von Willebrand factor propeptide and pathophysiological mechanisms in European and Iranian patients with type 3 von Willebrand disease enrolled in the 3WINTERS‐IPS study
Von Willebrand factor propeptide and pathophysiological mechanisms in European and Iranian patients with type 3 von Willebrand disease enrolled in the 3WINTERS‐IPS study Open
An increased VWFpp/VWF:Ag ratio is indicative of missense variants, whereas FVIII:C/VWF:Ag ratio does not discriminate missense from null alleles. The VWFpp level was not associated with the severity of bleeding phenotype.
View article: Efficacy and safety evaluation of Fanhdi<sup>®</sup>, a plasma‐derived factor VIII/ von Willebrand factor concentrate, in Von Willebrand's disease patients undergoing surgery or invasive procedures: A prospective clinical study
Efficacy and safety evaluation of Fanhdi<sup>®</sup>, a plasma‐derived factor VIII/ von Willebrand factor concentrate, in Von Willebrand's disease patients undergoing surgery or invasive procedures: A prospective clinical study Open
Von Willebrand's disease (VWD) is the most common hereditary blood-clotting disorder caused by a deficiency of von Willebrand Factor (VWF) and characterized by a defective platelet adhesion and aggregation.1 Replacement therapy with plasma…
View article: Genotypes of European and Iranian patients with type 3 von Willebrand disease enrolled in 3WINTERS-IPS
Genotypes of European and Iranian patients with type 3 von Willebrand disease enrolled in 3WINTERS-IPS Open
Type 3 von Willebrand disease (VWD3) is a rare and severe bleeding disorder characterized by often undetectable von Willebrand factor (VWF) plasma levels, a recessive inheritance pattern, and heterogeneous genotype. The objective of this s…
View article: An acquired factor V inhibitor induced uncontrolled bleeding in a postsurgery patient
An acquired factor V inhibitor induced uncontrolled bleeding in a postsurgery patient Open
This case report highlights the challenges in controlling bleeding and correcting coagulation tests through the use of bypassing agents in patients with FV inhibitors.
View article: Clinical characteristics and risk factors associated with COVID-19 severity in patients with haematological malignancies in Italy: a retrospective, multicentre, cohort study
Clinical characteristics and risk factors associated with COVID-19 severity in patients with haematological malignancies in Italy: a retrospective, multicentre, cohort study Open
Associazione italiana contro le leucemie, linfomi e mieloma-Varese Onlus.
View article: Unexpected Neurological Symptoms of Ruxolitinib: A Case Report
Unexpected Neurological Symptoms of Ruxolitinib: A Case Report Open
Ruxolitinib is a highly potent JAK2 inhibitor approved for the treatment of myelofibrosis (idiopathic or post-polycythemia vera or post-essential thrombocythemia) and, more recently, for polycythemia vera with an inadequate response to or …
View article: A phase III study comparing secondary long-term prophylaxis versus on-demand treatment with vWF/FVIII concentrates in severe inherited von Willebrand disease.
A phase III study comparing secondary long-term prophylaxis versus on-demand treatment with vWF/FVIII concentrates in severe inherited von Willebrand disease. Open
Despite the small sample size and the heterogeneity of the study population, patients on vWF/FVIII prophylaxis showed a reduction in bleeding risk and rate compared to on-demand treatment.
View article: PF802 ANTICOAGULANT THERAPY IN THROMBOCYTOPENIC PATIENTS WITH BLOOD CANCER AND CATHETER‐RELATED VTE: RESULTS FROM A DELPHI CONSENSUS ON PLATELETS COUNT BELOW 50 X 109L
PF802 ANTICOAGULANT THERAPY IN THROMBOCYTOPENIC PATIENTS WITH BLOOD CANCER AND CATHETER‐RELATED VTE: RESULTS FROM A DELPHI CONSENSUS ON PLATELETS COUNT BELOW 50 X 109L Open
Background: Management of venous thromboembolism (VTE) in patients with haematological malignancies (HM) and thrombocytopenia challenges clinicians because this scenario is encumbered with several risks. Our panel, composed by elected memb…
View article: Platelet cut-off for anticoagulant therapy in thrombocytopenic patients with blood cancer and venous thromboembolism: an expert consensus.
Platelet cut-off for anticoagulant therapy in thrombocytopenic patients with blood cancer and venous thromboembolism: an expert consensus. Open
We suggest using dose-adjusted LMWH according to PLT to optimise anticoagulant treatment in patients at high bleeding risk.
View article: Current challenges in the diagnosis and management of patients with inherited von Willebrand's disease in Italy: an Expert Meeting Report on the diagnosis and surgical and secondary long-term prophylaxis.
Current challenges in the diagnosis and management of patients with inherited von Willebrand's disease in Italy: an Expert Meeting Report on the diagnosis and surgical and secondary long-term prophylaxis. Open
Recent advances in the care of von Willebrand's disease (vWD) have allowed the majority of patients to be managed adequately. Even in the more severe forms, it is now possible to control recurrent bleeding through secondary long-term proph…
View article: Pathophysiology and General Management of Patients with Massive Blood Loss
Pathophysiology and General Management of Patients with Massive Blood Loss Open
1Hematology and Transfusion Medicine, L. Sacco University Hospital, Department of Oncology and Hematology Oncology, University of Milan 2Department of Transfusion Medicine, Fondazione IRCCS Cá Granda Ospedale Maggiore Policlinico, Milan, I…
View article: Massive bleeding
Massive bleeding Open
Hematology and Transfusion Medicine, L. Sacco University Hospital, Department of Oncology and Hematology Oncology, University of Milan, Italy This is an open access Abstract Book distributed under the Attribution-NonCommercial-NoDerivs (CC…
View article: Fifth Åland Island conference on von Willebrand disease
Fifth Åland Island conference on von Willebrand disease Open
The fifth Åland Island meeting on von Willebrand disease ( VWD ) was held on the Åland Islands, Finland, from 22 to 24 September 2016—90 years after the first case of VWD was diagnosed in a patient from the Åland Islands in 1926. This meet…
View article: Diagnosis and management of von Willebrand disease in Italy
Diagnosis and management of von Willebrand disease in Italy Open
Despite the outstanding progress in understanding the clinical and pathophysiological aspects of von Willebrand disease (VWD), the general awareness about its true prevalence and heterogeneity of clinical features remain still incomplete. …
View article: Activated prothrombin complex concentrate (<scp>FEIBA</scp><sup>®</sup>) in acquired haemophilia A: a large multicentre Italian study – the <scp>FAIR</scp> Registry
Activated prothrombin complex concentrate (<span>FEIBA</span><sup>®</sup>) in acquired haemophilia A: a large multicentre Italian study – the <span>FAIR</span> Registry Open
Acquired haemophilia A (AHA) is a rare bleeding disorder caused by the spontaneous development of auto-antibodies against coagulation factor VIII (FVIII) in males and females with previously normal haemostasis (Kessler & Knobl, 2015). This…