Fanconi's anaemia associated with haemophilia A

• Fanconi's anaemia and haemophilia A are born inherited diseases creating haemostatic defects. The association of these two rare diseases in one patient is described. The patient's haemophilia was studied with a newly developed immunological technique determining the plasma antigen associated with Factor VIII activity, and was found to be a genetic variant of moderately severe haemophilia A. It was not possible to demonstrate a common bone marrow defect or a common immunological or genetical background of the two diseases. The double haemostatic defect created, i.e. Factor VIII deficiency and thrombocytopenia, resulted in only a slight increase in bleeding tendency. A favourable result was obtained with corticosteroid and androgenic treatment.

Nyckelord

Abnormalities, Multiple

Anemia, Aplastic

Child

Factor VIII

Fanconi Anemia

Hemophilia A

Humans

Male

Thrombocytopenia

Case Reports

Journal Article

Publikations- och innehållstyp

art (ämneskategori)

ref (ämneskategori)