Získaná hemofilie A Dotaz Zobrazit nápovědu
Acquired haemophilia A is a rare auto-immune disease caused by an inhibitory antibody to factor VIII. Patients with this disorder are at high risk of severe bleeding until the inhibitor has been eradicated. Management of this disorder consists in rapid accurate diagnosis, control of bleeding and eradication of the inhibitor by immunosuppression. The cessation of bleeding is based mainly on recombinant factor VIIa and activated prothrombin complex concentrate which are approximately equally efficacious. Immunosuppression is still based on steroids alone or with combination with cyclopfosphamide which may result in a higher rate of remission. New drugs as rituximab or cyclosporine A are the second line option. In case of life-threatening bleeding immunoadsorption and high dose of fac-tor VIII could be advantageous.
- MeSH
- autoprotilátky analýza MeSH
- faktor VIII imunologie MeSH
- hemofilie A komplikace diagnóza imunologie terapie MeSH
- krvácení etiologie terapie MeSH
- lidé MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH
- přehledy MeSH
- Názvy látek
- autoprotilátky MeSH
- faktor VIII MeSH
Case report of a 74-year-old male with acquired hemophilia A, whose etiology was not found, confirmed by hemocoagulation examinations, with clinical manifestation of protracted macroscopic hematuria, spontaneous skin and intramuscular bleedings. Treatment comprised conservative therapy administration of hemostyptics, red blood cells transfusions application of recombinant human factor VII (rFVII) and immunosuppression. Finally, patient died due to nosocomial infection caused by multidrug-resistant pulmonary pathogen.Key words: acquired hemophilia A - bleeding.
- MeSH
- hematurie etiologie MeSH
- hemofilie A * komplikace diagnóza etiologie MeSH
- krvácení etiologie MeSH
- lidé MeSH
- senioři MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- MeSH
- autoimunitní hemolytická anemie MeSH
- dospělí MeSH
- hemofilie A MeSH
- krevní nemoci * diagnóza terapie MeSH
- leukemie MeSH
- lidé středního věku MeSH
- lidé MeSH
- následné studie MeSH
- perniciózní anemie MeSH
- prognóza MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH