Mild Haemostatic Problems Associated with Congenital Heterozygous α2-Antiplasmin Deficiency

F W G Leebeek; J Stibbe; E A R Knot; C Kluft; M J Gomes; M Beudeker

doi:10.1055/s-0038-1646773

Thrombosis and Haemostasis, Table of Contents

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

Authors

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

Abstract

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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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References

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