Registry of inherited coagulopathies in Brazil: first report

Abstract

Inherited coagulopathies are bleeding disorders, which require treatment for life. Keeping an updated registry on these diseases is crucial for planning care, documenting prevalence of diseases and evaluating effectiveness of resources. We have analysed data from 26 treatment centres on coagulopathies in Brazil. Information included socio-demographic data, diagnosis of coagulopathies, severity of haemophilias A and B, presence and quantification of inhibitors in haemophilia, type of von Willebrand disease (VWD) and infection status for viral diseases. On 1 July 2007, there were 10 982 patients with inherited coagulopathies in Brazil, of which 6881 (62.7%) corresponded to haemophilia A, 1291 (11.7%) to haemophilia B, 2333 (21.2%) to VWD, 258 (2.4%) to other coagulopathies and 219 (2.0%) to undiagnosed bleeding disorders. Haemophilia A and B inhibitors were present in 9.9% and 1.9% of the patients, respectively. Human immunodeficiency virus infection was present is 6.5%, 4.8% and 1% of patients with haemophilia A, B and VWD, respectively. Hepatitis C virus infection was present in 34.9%, 29.7% and 12% of patients with haemophilia A, B and VWD, respectively. Infection by hepatitis B and human T-cell leukemia-lymphoma virus was also reported. This is the first report on the registry of patients with inherited coagulopathies in Brazil, supposed to be the third largest population of patients with haemophilia.

Identification and prevalence of von Willebrand disease type 2N (Normandy) in Australia

“…4 [15][16][17][18][19][20][21][22]. In total, type 2N VWD represents 1-2% of all local VWD presentations.…”

“…Also, consanguinity and lack of comprehensive testing facilities often leads to identification of primarily the severest forms of VWD, leading to a greater relative prevalence of type 3 VWD, which is otherwise the rarest form of VWD in western regions. [17] and from the Emilia-Romagna Registry of Italy [18,19]), North America (Canadian Hemophilia Registry [20]), South America (Argentina [21] and Brazil [22]), and India [16]. (a) Prevalence considering and showing all VWD types.…”

“…Types 2A and 2M are generally the most common presentations, though these are also sometimes underrecognized when available test processes may not permit their differential diagnosis (e.g. Argentina [21], Brazil [22] and China [15]). Types 2B and 2N VWD are less common phenotypes, although it is likely that their prevalence may also be underrecognized, given either the lack of available testing or specific investigations.…”

Influence of class I and II HLA alleles on inhibitor development in severe haemophilia A patients from the South of Brazil

“…The incidence rate of haemophiliacs across all ethnic groups is approximately 1:10 000 male births. Currently, in Brazil, there are 8172 (62.7%) haemophilia A patients and 1291 (11.7%) haemophilia B patients and 705 of these patients have developed inhibitors [2]. The prevalence of the anti‐FVIII inhibitors indicated in the previous literature is between 5% and 7% in patients with mild/moderate haemophilia and between 12% and 13% in patients with severe haemophilia A [3].…”

Prevalence and risk factors associated with hepatitis C among Brazilian male patients with haemophilia: A long‐term follow‐up

“…Similar to what happened in developed countries, improvements in the health care of these patients were disseminated in Brazil by the implementation of a nationwide regional network of blood banks that act as referral centres for the treatment of haemophilia. These centres were crucial for carrying out blood safety procedures and providing home infusion and prophylaxis treatments, which greatly contributed to reduce the incidence of HCV infection in younger patients with haemophilia . The results of blood safety public health policies were clearly demonstrated in our study.…”