Rapporto ISTISAN 20/14 - National Registry of congenital bleeding disorders. Report 2018. Francesca Abbonizio, Romano Arcieri, Associazione Italiana Centri Emofilia (AICE) e Adele Giampaolo - ISS (EN)

Istituto Superiore di Sanità

National Registry of congenital bleeding disorders. Report 2018.
Francesca Abbonizio, Romano Arcieri, Associazione Italiana Centri Emofilia (AICE) and Adele Giampaolo
2020, iii, 53 p. Rapporti ISTISAN 20/14 (in Italian)

The National Registry of congenital bleeding disorders collects data on number and distribution of patients with bleeding disorders, with special attention to the epidemiologic surveillance of complications and to the evaluation of needs of drugs necessary to treatment. Data of the Registry 2018 are from 53/54 Italian Hemophilia Centers and refer to 10,554 people with congenital bleeding disorders: 4,109 with Hemophilia A, 882 with Hemophilia B, 3,245 with von Willebrand disease and 2,318 with defects of other coagulation factors. In the analyzed patients, 234 are HIV-positive, but no new case recorded in the last 30 years; HCV-positive patients are 1,510 in total. Collected therapeutic plans referring to the year 2018 were relative to 54.2% of patients with severe hemophilia A and 53.2% of patients with severe Hemophilia B. Based on therapeutic plans, patients recorded with current inhibitors during 2018 are 81: 85.2% with severe Hemophilia A. Factor VIII prescribed for Hemophilia A was 380,000,000 International Units (IU), 82.6% was recombinant FVIII. Factor IX prescribed for Hemophilia B therapy was 39,400,000 IU.
Key words: Haemophilia centres; Bleeding disorders; Haemophilia; Factor VIII; Plasma-derived products; Recombinant clotting factors