Publications by authors named "Roberto Mario Santi"
Article Synopsis
- Lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) is a rare autoimmune disorder linked to antiphospholipid syndrome, which causes severe bleeding due to low prothrombin levels, as illustrated by a case study of a 34-year-old woman with heavy menstrual bleeding and related complications.
- The patient underwent extensive testing, revealing deficiencies in factors II and IX, leading to the initiation of immunosuppressive treatment with Rituximab and hormonal therapy with desogestrel to manage antibody levels and reduce bleeding.
- After one year of treatment, the patient achieved complete remission, with restored levels of prothrombin and factor IX while showing reduced antibody
Article Synopsis
- - Plasma-derived von Willebrand factor-containing factor VIII concentrates (pd-VWF/FVIII-C), specifically Fanhdi® and Alphanate®, are commonly used to treat von Willebrand disease (VWD) in Italy, and a study evaluated their real-world effectiveness and safety.
- - The retrospective study involved 57 patients with different types of VWD, treating various bleeding episodes, surgical procedures, and long-term prophylaxis over a period from 2007 to 2019.
- - Results showed that pd-VWF/FVIII-C had excellent or good efficacy in 85% of bleeding episodes, 98% of surgeries, and 100% of long-term prophylaxis cases, with no serious safety concerns reported. *
Background: Hemophilic arthropathy is a detrimental condition that crucially affects functional outcomes in hemophilic patients. In recent years, due to the advances in systemic therapies, growing attention has been raised in the rehabilitation field in order to improve functional outcomes of hemophilic patients. However, the optimal rehabilitation modalities in these patients are far from being fully characterized.
View Article and Find Full Text PDFThrombosis in multiple myeloma: risk stratification, antithrombotic prophylaxis, and management of acute events. A consensus-based position paper from an expert panel.
Haematologica
November 2022
The introduction of new therapeutic agents for multiple myeloma (MM), including proteasome inhibitors, immunomodulatory drugs, and monoclonal antibodies, has improved the outcomes of patients but, in parallel, has changed the frequency and epidemiology of thrombotic events. Thrombosis is now a significant cause of morbidity and mortality in MM patients, and optimal thromboprophylaxis is far from being reached. Moving from the recognition that the above issue represents an unmet clinical need, an expert panel assessed the scientific literature and composed a framework of recommendations for improving thrombosis control in patients who are candidates for active treatment for MM.
View Article and Find Full Text PDFHaemophilic Pelvic Pseudotumour: A New Surgical Option.
Healthcare (Basel)
September 2021
Background: Haemophilia is an inherited coagulopathy caused by the absence or dysfunction of clotting factor VIII or IX. Clinical manifestations are generally secondary to recurrent bleeding episodes mainly in the musculoskeletal system. Bleeding symptoms appear early in life and, when the disease is severe (when plasma factor VIII or IX activity is <1% of normal), joint and muscle bleeding may occur spontaneously.
View Article and Find Full Text PDFCurrent data suggests that the risk of venous thromboembolism (VTE) in patients with non-Hodgkin lymphoma (NHL) is comparable to that observed in patients with solid tumours, although more robust confirmatory analyses are required. With that in mind, we investigated the occurrence of VTE in a pooled analysis of 12 "Fondazione Italiana Linfomi" (FIL) prospective clinical studies. Specifically, we wished to assess the cumulative incidence of VTE in NHL patients, evaluate the predictive value of the Khorana Score (KS), and identify other potential risk factors for VTEs.
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