Publications by authors named "Roberto 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
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  • - 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. *
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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.

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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.

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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.

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Immune thrombocytopenia (ITP) secondary to chronic lymphoproliferative disorders (LPDs) is poorly responsive to conventional treatments. We conducted a multicenter phase 2 prospective 24-week study in 18 patients with ITP secondary to LPDs to assess the safety and efficacy of eltrombopag. Responsive patients entered an extension study for up to 5 years.

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Background: Management of venous thromboembolism (VTE) in patients with haematologic malignancies and thrombocytopenia is clinically challenging due to the related risks. No prospective studies or clinical trials have been carried out and, therefore, no solid evidence on this compelling issue is available.

Methods: Given this, an expert panel endorsed by the Gruppo Italiano Malattie Ematologiche dell'Adulto Working Party on Thrombosis and Haemostasis was set up to produce a formal consensus, according to the RAND method, in order to issue clinical recommendations about the platelet (PLT) cut-off for safe administration of low molecular weight heparin (LMWH) in thrombocytopenic (PLT <100×10/L) adult patients with haematologic malignancies affected by acute (<1 month) or non-acute VTE.

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Current 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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Article Synopsis
  • The direct oral anticoagulants (DOACs) have been used since 2009 to prevent stroke in atrial fibrillation and manage venous thromboembolism, offering alternatives to Vitamin K antagonists.
  • The four DOACs include three factor Xa inhibitors (rivaroxaban, apixaban, edoxaban) and one direct thrombin inhibitor (dabigatran), but proper knowledge of their pharmacokinetics and pharmacodynamics is crucial for internists to ensure safe usage and management of side effects.
  • A consensus paper from Italian medical societies provides guidance for using DOACs in various settings, focusing on protocols for elderly patients with complex health issues, and addressing concerns like drug interactions and emergency procedures.
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Objectives: Recent evidences suggest that the activation of peroxisome proliferator-activated receptor (PPAR)-gamma2, which plays an important role in vascular homeostasis, also regulates the expression of the Receptor for Advanced Glycation End products (RAGE). In turn, low levels of soluble RAGE (sRAGE) have recently emerged as a valuable biomarker of vascular inflammation. The potential alterations in sRAGE concentrations in peripheral arterial disease (PAD), however, have not been yet investigated.

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Titanium tetrachloride reacts with 2,6-bis[(1-phenylimino)ethyl]pyridine, 1, and 2,6-bis[1-(2,6-diisopropylphenylimino)ethyl]pyridine, 2, giving the adducts of general formulae [Ti1Cl3]Cl, 3, and [Ti2Cl3]Cl, 6, the latter through the intermediacy of the covalently bonded [Ti2Cl4], 4. Heating 6 leads to reduction to the titanium(III) derivative [Ti2Cl3], 12, the latter characterized by X-ray diffraction methods. The reaction of [Ti1Cl3]Cl with a toluene solution of MAO proceeds with methylation at the ortho-position of the pyridine ring to give the titanium(iv) derivative [Ti(C22H21N3)Cl3], 8.

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The reactions of dialkyl sulfones [R(2)SO(2): R = Me, Et, Ph, R(2)=-(CH(2))(4)-] with the metal tetrachlorides of Group 4 [MCl(4): M = Ti, Zr, Hf] give different products mainly depending on the sulfone/M molar ratio. Compounds of formula [M(2)Cl(8)(R(2)SO(2))(2)][M = Ti, R(2)=-(CH(2))(4)-; M = Zr, R = Et, R = Ph] and [MCl(4)(R(2)SO(2))(2)](sulfone/M = 2)[M = Ti, R = Me; M = Zr, R = Me, R = Ph, R(2)=-(CH(2))(4)-; M = Hf, R = Me, R(2)=-(CH(2))(4)-] have been obtained. By X-ray diffraction methods the dinuclear titanium and zirconium adducts, [Ti(2)Cl(8)(mu-sulfolane-O,O')(2)] and [Zr(2)Cl(8)(mu-Ph(2)SO(2)-O,O')(2)] have been established to contain bridging sulfone and hexacoordinated metal centres, while the mononuclear zirconium complex [ZrCl(4)(Me(2)SO(2))(2)] has cis-monodentate sulfones in a slightly distorted octahedral geometry.

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