Hemophilia

Česká verzia

Authors: P. Smejkal; M. Matýšková; M. Penka
Authors place of work: Oddělení klinické hematologie FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Miroslav Penka, CSc.
Published in the journal: Vnitř Lék 2008; 54(10): 992-999
Category: Reviews

Summary

Hemophilia is the most spread hereditary bleeding disorder with severe bleeding symptoms. Although the number of hemophiliacs is below 0.1‰ in population, the care of these patients consumes a lot of financial expenditures, especially for treatment in appearance of inhibitor. Management of hemophilia is best provided by specialist in a hemophilia treatment centre. However, all physicians can meet the patients with hemophilia in urgent situation or with their chronic problems. The aim of this article is to give an overview of this disease from pathophysiology, clinical manifestation and diagnosis (including prenatal) to treatment. We describe most frequent complications of hemophilia treatment, particularly management of inhibitor.

Key words:
hemophilia – diagnosis – treatment – F VIII inhibitor – immune tolerance – guidelines

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