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Case Reports
. 2020 Sep;31(6):397-401.
doi: 10.1097/MBC.0000000000000908.

Hemophilia A and von Willebrand deficiency: therapeutic implications

Maria Basso  1 , Ilaria Lazzareschi  2   3 , Antonietta Curatola  2 , Leonardo Di Gennaro  1 , Danilo Buonsenso  2   3 , Antonio Gatto  2 , Erica De Candia  1   4 , Raimondo De Cristofaro  1   4
Affiliations
Case Reports

Hemophilia A and von Willebrand deficiency: therapeutic implications

Maria Basso et al. Blood Coagul Fibrinolysis. 2020 Sep.

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.

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