Publication at Faculty of Medicine in Hradec Králové |
2022
Abstract
Thrombotic thrombocytopenic purpura is a rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenia and tissue ischemia caused by congenital or acquired deficiency od ADAMTS13 (A disintegrinlike and metalloprotease [reprolysintype] with thrombospondin type 1 motif 13) enzyme. Rapid diagnosis including determination of ADAMTS13 activity is crucial for the early beginning of the treatment.
Current therapeutical options include therapeutic plasma exchange and in the immune thrombotic thrombocytopenic purpura the combination of immunosuppressive therapy with caplacizumab, a humanized antibody against A1 domain of von Willebrand factor.