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Thromb Haemost 1988; 59(01): 096-100
DOI: 10.1055/s-0038-1646773
Review Article
Schattauer GmbH Stuttgart

Mild Haemostatic Problems Associated with Congenital Heterozygous α2-Antiplasmin Deficiency

F W G Leebeek
*   The Department of Haematology of the University Hospital Dijkzigt, Rotterdam, The Netherlands
,
J Stibbe
*   The Department of Haematology of the University Hospital Dijkzigt, Rotterdam, The Netherlands
,
E A R Knot
**   The Department of Internal Medicine II of the University Hospital Dijkzigt, Rotterdam, The Netherlands
,
C Kluft
***   Gaubius Institute TNO, Leiden, The Netherlands.
,
M J Gomes
*   The Department of Haematology of the University Hospital Dijkzigt, Rotterdam, The Netherlands
,
M Beudeker
*   The Department of Haematology of the University Hospital Dijkzigt, Rotterdam, The Netherlands
› Institutsangaben
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Publikationsverlauf

Received 01. April 1987

Accepted after revision 20. Oktober 1987

Publikationsdatum:
18. April 2018 (online)

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Summary

A Dutch family, of which 13 members are heterozygotes, deficient for α2-antiplasmin (α2-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 α2-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 α2-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 α2-AP is due to a decreased synthesis of a normal α2-AP molecule. This present study brings the frequency of heterozygous α2-AP deficient patients with a bleeding tendency to 13 out of 59 heterozygotes reported in the literature.

Key words

Fibrinolysis - α2-antiplasmin - Deficiency - Plasminogen - Haemorrhagic diathesis
 

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