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Inherited platelet abnormalities associated with low factor VIII activity in the same family.
- Source :
-
Acta haematologica [Acta Haematol] 1977 Mar; Vol. 57 (4), pp. 225-32. - Publication Year :
- 1977
-
Abstract
- Six of eight examined members belonging to two generations of the same (NEG-TUR) family were shown to have functional changes in platelets and/or a moderate decrease of factor VIII activity (FVIII:C) in plasma, with normal values of factor VIII-related antigen (VIII R:AG). Platelet defects (mainly a reduced PF3 availability, present in five patients) and factor VIII decrease were combined differently in individual members. Only two male members with both the PF 3 and FVIII:C defects had moderate haemorrhagic symptoms following traumatic injuries. One of them had also an absent adhesiveness to glass, the other one an absent adhesiveness to collagen and a reduced platelet aggregation by ADP and by collagen. Bleeding time, platelet function tests (in the other members), and routine coagulation tests were within normal range; ristocetin aggregation was also normal in all members. We think that two inherited defects, a mild haemophilia A and a "sui generis" thrombocytopathy, co-exist in this family.
Details
- Language :
- English
- ISSN :
- 0001-5792
- Volume :
- 57
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Acta haematologica
- Publication Type :
- Academic Journal
- Accession number :
- 403728
- Full Text :
- https://doi.org/10.1159/000207885