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. 2017 Mar 19;6(3):34.
doi: 10.3390/jcm6030034.

Italian Registry of Congenital Bleeding Disorders

Adele Giampaolo  1 , Francesca Abbonizio  2 , Romano Arcieri  3 , Hamisa Jane Hassan  4
Affiliations

Italian Registry of Congenital Bleeding Disorders

Adele Giampaolo et al. J Clin Med. .

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.

Keywords: Hemophilia A; Hemophilia B; bleeding disorders; clotting factors; rare diseases; registry.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Trend in the total number of patients with bleeding disorders registered in the National Registry of Congenital Coagulopathies (NRCC) from 2011 to 2015. HA: Hemophilia A; HB: Hemophilia B.
Figure 2
Figure 2
Patients with inherited bleeding disorders recorded in the NRCC-2015. * “Other patients” include HA and HB carriers and patients with congenital platelet disorders. vWD: von Willebrand Disease.
Figure 3
Figure 3
Trend of estimated FVIII used in Italy over the years based on the entire population of severe HA.
See this image and copyright information in PMC

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