Congenital Deficiency of α2-Plasmin Inhibitor in Three Sisters
- 1 January 1982
- journal article
- research article
- Published by S. Karger AG in Pathophysiology of Haemostasis and Thrombosis
- Vol. 11 (3), 176-184
- https://doi.org/10.1159/000214659
Abstract
3 young Japanese sisters with congenital α2-plasmin inhibitor (α2-PI) deficiency are reported. They have mild umbilical bleeding and/or repeated prolonged bleeding after minor trauma, but rarely spontaneous bleedings. The most characteristic hemostatic findings were shortened whole blood clot lysis time and euglobulin lysis time. Activities of all hemostatic factors except α2-PI were within normal range. Both functional and immunological absence of α2-PI were found in the plasma, and this failure to detect α2-PI was not corrected by the addition of the patient’s plasma of the first described case of α2-PI deficiency. Clinical and laboratory data revealed that these patients were probably homozygous for α2-PI deficiency and born of heterozygous parents, but not of consanguineous ones. Bleeding episodes due to deficiency of (α2-PI in these patients were well controlled by an antifibrinolytic agent, tranexamic acid.Keywords
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