Heamophilia and treatment of inhibitor
Authors:
V. Hampalová
Authors place of work:
Dětské oddělení, Uherskohradišťská nemocnice a. s., Uherské Hradiště
Published in the journal:
Čes-slov Pediat 2018; 73 (7): 455-458.
Category:
Case Report
Summary
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
Zdroje
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.
2. Blatný J, Ovesná P. Léčba dětí s hemofilií v České republice. Čes-slov Pediat 2017; 72 (3): 148–153.
3. Blatný J, Hrachovinová I, Hrdličková R, et al. Diagnostika a léčba hemofilie. Český národní hemofilický program 2012. http://cnhp.registry.cz/res/file/konference/2012/diagnostika-lecba-hemofilie.pdf.
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.
5. Hay CR, DiMichele DM. The principal results of the International Immune Tolerance Study: a randomized dose comparison. Blood 2012; 119: 1335–1344.
6. Salaj P, Smejkal P, Komrska V, a spol. Standardy péče o nemocné s hemofilií. Český národní hemofilický program 2012 (1. rev.). http://cnhp.registry.cz/res/file/guidelines/2012-standardy-pece-o-nemocne-s-hemofilii.pdf.
Štítky
Haematology Neonatology Paediatrics General practitioner for children and adolescentsČlánok vyšiel v časopise
Czech-Slovak Pediatrics
2018 Číslo 7
- What Effect Can Be Expected from Limosilactobacillus reuteri in Mucositis and Peri-Implantitis?
- International Recommendations for COVID-19 Vaccination in People with Bleeding Disorders
- Administration of aPCC as a Prevention of Bleeding After Major Cardiac Surgical Procedures
- Position of aPCC in the Treatment of Hemophilia A Complicated by the Development of Inhibitors
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