Abstract |
A Dutch family, of which 13 members are heterozygotes, deficient for alpha 2-antiplasmin (alpha 2-AP) is reported. Clinical studies showed that 2 heterozygotes had a mild bleeding tendency, which presented as bleeding episodes after tooth extraction and after surgery and, in one patient, also as excessive menstruation. Laboratory investigations revealed an alpha 2-AP activity of 62% (51-71) (median and range) and an antigen level of 60% (60-66). The plasminogen binding as well as the fibrin binding properties of alpha 2-AP were normal. Plasminogen concentrations were significantly higher in the heterozygotes compared to the other family members. However, free plasminogen not bound to histidine-rich glycoprotein was not significantly different between these two groups. We propose that in this family the deficiency of alpha 2-AP is due to a decreased synthesis of a normal alpha 2-AP molecule. This present study brings the frequency of heterozygous alpha 2-AP deficient patients with a bleeding tendency to 13 out of 59 heterozygotes reported in the literature.
|
Authors | F W Leebeek, J Stibbe, E A Knot, C Kluft, M J Gomes, M Beudeker |
Journal | Thrombosis and haemostasis
(Thromb Haemost)
Vol. 59
Issue 1
Pg. 96-100
(Feb 25 1988)
ISSN: 0340-6245 [Print] Germany |
PMID | 3363537
(Publication Type: Case Reports, Journal Article)
|
Chemical References |
- alpha-2-Antiplasmin
- Plasminogen
|
Topics |
- Adult
- Female
- Fibrinolysis
- Genetic Carrier Screening
- Hemostasis
- Heterozygote
- Humans
- Pedigree
- Plasminogen
(metabolism)
- alpha-2-Antiplasmin
(deficiency)
|