Heamophilia and treatment of inhibitor
Authors:
V. Hampalová
Authors‘ workplace:
Dětské oddělení, Uherskohradišťská nemocnice a. s., Uherské Hradiště
Published in:
Čes-slov Pediat 2018; 73 (7): 455-458.
Category:
Case Report
Overview
This case report presents two cases (boys) of severe haemophilia A. After repeated doses of deficient coagulation factor (FVIII) inhibitors (antibody) have been detected. The presence of inhibitors significantly impairs treatment of haemophilia since it causes decreased treatment efficacy and increased probability of severe bleeding. A possible solution might be the immune tolerance treatment (ITT) with often high success rate. To improve patients’ comfort during ITI treatment with frequent intravenous applications, it is almost necessary to place a venous port catheter which also helps to decrease potential risk of infectious complications. First boy has been successfully treated with ITI, whereas parents of the second boy have been refusing venous port implantation as well as ITT treatment itself.
KEY WORDS:
haemophilia, bleeding, prophylaxis, inhibitor, immunotolerance, factor VIII/IX, FVIII, FIX
Sources
1. Blatný J, Komrska V, Blažek B, et al. Inhibitors incidence rate in Czech previously untreated patients with haemophilia A has not increased since introduction of recombinant factor VIII treatment in 2003. Blood Coagulation and Fibrinolysis 2015; 26 (6): 673–678.
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4. Collins PW, Chalmers E, Hart DP, et al. Diagnosis and treatment of factor VIII and IX inhibitors in congenital haemophilia (4th edition). Br J Haematol 2013; 160: 153–170. doi: 10.1111/bjh.12091.
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Labels
Haematology Neonatology Paediatrics General practitioner for children and adolescentsArticle was published in
Czech-Slovak Pediatrics
2018 Issue 7
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