Hemophilia A and von Willebrand deficiency: therapeutic implications

Ilaria Lazzareschi, Erica De Candia, Raimondo De Cristofaro, Maria Basso, Antonietta Curatola, Leonardo Di Gennaro, Danilo Buonsenso

Risultato della ricerca: Contributo in rivistaArticolo in rivista

Abstract

Hemophilia A is an X-linked bleeding disorder caused by a deficiency of factor VIII. Depending on the factor VIII activity in patient's plasma, we can have three different forms of hemophilia A: mild (5-40 IU/dl), moderate (1-5 IU/dl) and severe (<1 IU/dl). The most common symptoms include recurrent bleeding episodes of soft tissues and joints. The treatment is based on the prophylactic use of clotting factor concentrates to prevent bleeding episodes. We describe three cases of patients with initially diagnosis of hemophilia A that show different clinical severity, undergoing prophylactic therapies with low benefit. In these patients, the dosage of von Willebrand antigen revealed either low level or absence of this factor, which in one case was caused by the occurrence of a type III form of von Willebrand disease
Lingua originaleEnglish
pagine (da-a)N/A-N/A
RivistaBLOOD COAGULATION &amp; FIBRINOLYSIS
DOI
Stato di pubblicazionePubblicato - 2020

Keywords

  • VonWillebrand factor
  • factor VIII
  • hemophilia

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