• OJHM vol. 3 num. 1 - Original Article. Unmet needs and perceived shortcomings due to covid-19 in hemophilia patients

    The large allocation of human and economic resources to face the pandemic and the lockdown mobility restrictions led the community of persons with haemophilia to be resilient and adaptable, engaging in adopting new methods to ensure their continuity of care. This study aimed to evaluate the emotions which have affected the haemophilia adult patient's daily life, during the first year of pandemic and what behaviors have been adopted to face the socio economic re strictions including lockdown, social distancing and self isolation, as well as the limited access to Hemophilia Treatment Centres (HCT).

  • OJHM vol. 3 num. 1 - Congress Abstract. Prophylaxis in von Willebrand Disease patients.

    G. Sottilotta

    Von Willebrand disease (VWD) is the most common inherited bleeding disorder resulting from a deficiency or dysfunction of von Willebrand factor (VWF). VWF has an integral role in hemostasis because it binds to and stabilizes FVIII as well as facilitating platelet adhesion to the injured endothelium. VWD has been characterized into three types: type 1, the most
    common, caused by a partial quantitative deficiency of VWF; type 2 which is observed in approximately 20% of cases and results from VWF dysfunction due to qualitative abnormalities; type 2 VWD has been further subdivided into four subtypes: while type 2A is characterized by a loss of high-molecular weight VWF, type 2B results from a change in the VWF structure leading to an increased affinity to platelets. 

  • OJHM vol. 3 num. 1 - Congress Abstract. The role of the nurse in the hospital and home management of the hemophiliac patient

    O. Di Gregorio

    The transformation of haemophilia from a life threatening disease into a long-term manageable condition is changing the role of the haemophilia nurse specialist It will likely be less clinically focused and have a greater emphasis on psychosocial support, including motivation, shared decision making, support and self-advocacy for patients. Placing the person in all his complexity at the center of the treatment process is the main objective that the entire health system must achieve: it is no longer possible to reason in pharmacocentric terms, but the patient's needs must be given priority.

  • OJHM vol. 3 num. 1 - Congress Abstract. Thrombotic microangiopathies: thrombotic thrombocytopenic purpura vs atypical hemolytic uremic syndrome

    L. Fianchi

    Thrombotic microangiopathies (TMAs) are rare and life threatening diseases. In recent years, there have been major advances in understanding the pathophysiology, classification, and treatment of these disorders, including the introduction of new drugs. Early differential diagnosis is crucial for prompt treatment to reduce high mortality rates and late organ damage.

  • OJHM vol. 3 num. 1 - Congress Abstract. Factor XIII deficiency: clinical manifestations and treatment

    E. Zanon

    Background: FXIII deficiency is a very rare coagulation disorder Bleeds are usually muscular or mucocutaneous, but bleeding from umbilical cord at birth and intracranial hemorrhages (ICHs) can occur in cases of severe disease Plasma derived concentrates (Fibrogammin ®) and RFXIII concentrates (Novothirteen ®) can be used on demand to control the bleeding. The best treatment in patients with severe disease is the use of plasma derived or recombinant FXIII concentrates on prophylaxis at the dose of 10 26 IU/kg every 4 6 weeks and 35 IU/kg every 4 weeks, respectively Few data on the use of rFXIII in the real world scenario are available.