Abstract
In Italy, the surveillance of people with bleeding disorders is based on the National Registry of Congenital Coagulopathies (NRCC) managed by the Italian National Institute of Health (Istituto Superiore di Sanità). The NRCC collects epidemiological and therapeutic data from the 54 Hemophilia Treatment Centers, members of the Italian Association of Hemophilia Centres (AICE). The number of people identified with bleeding disorders has increased over the years, with the number rising from approx. 7000 in 2000 to over 11,000 in 2015. The NRCC includes 4020 patients with hemophilia A and 859 patients with hemophilia B. The prevalence of the rare type 3 vWD is 0.20/100,000 inhabitants. Less common congenital bleeding disorders include the following deficiencies: Factor I (fibrinogen), Factor II (prothrombin), Factor V, Factor VII, Factor X, Factor XI and Factor XIII, which affect 1953 patients. Hepatitis C Virus (HCV) infection affects 1561 patients, more than 200 of whom have two infections (HCV + HIV). Estimated hemophilia-related drug consumption in 2015 was approx. 550 million IU of FVIII for hemophilia A patients and approx. 70 million IU of FIX for hemophilia B patients. The NRCC, with its bleeding disorder data set, is a tool that can provide answers to fundamental questions in public health, monitoring care provision and drug treatment, as well as facilitating clinical and epidemiological research.
Highlights
Blood coagulation is a complex mechanism that is required for the rapid establishment of a stable fibrin clot
We provide epidemiological information on patients with bleeding disorders as well as an estimate of FVIII and FIX products required in Italy to treat HA and HB patients using data collected in 2015 by the Italian registry of bleeding disorders
Data are collected on the basis of the therapeutic plans, which are mandatory by law and indicate the diagnosis of the bleeding disorder, its severity, treatment regimen, time covered by the therapeutic plan, dosage, source and brand name of the product, and the total number of International Units (IU) assigned
Summary
Blood coagulation is a complex mechanism that is required for the rapid establishment of a stable fibrin clot. A series of interdependent enzyme-mediated reactions translate the molecular signals that initiate blood coagulation into the formation of the fibrin clot. Congenital coagulopathies result when there is a deficiency of protein cofactors and enzymes implicated in blood coagulation [1]. Less common congenital bleeding disorders include deficiencies of Factor I (fibrinogen), Factor II (prothrombin), Factor V, Factor VII, Factor X, Factor XI and Factor XIII [1]. Therapy consists of replacing the deficient clotting factor through the administration of the specific factor concentrate; when the specific factor concentrates are not available, multi-factor concentrates, such as prothrombin complex concentrates, or human plasma (fresh-frozen plasma or commercially available products) can be used
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