A bleeding disorder due to deficiency of alpha 2-antiplasmin.

A bleeding disorder due to deficiency of a , 2 - antiplasmin

a2-Antiplasmin is a plasma proteinase inhibitor with an estimated molecular weight of 65 000-70000 daltons. It is a potent inhibitor of plasmin, its covalent binding to fibrin being mediated by factor XIIIa. Bound to fibrin, a2-antiplasmin reduces plasmin mediated fibrinolysis. Inherited lack of a2antiplasmin leads to increased fibrinolysis and a haemorrhagic diathesis. There are reports of con...

Acquired alpha 2 antiplasmin deficiency in glomerular proteinuria.

Isolated antiplasmin deficiency presenting as a spontaneous bleeding disorder in a 63-year-old man.

Spontaneous bleeding in adults is a major problem, and in a significant number of these patients no cause is found. A 63-year-old Caucasian man presented to our hematology clinic with a large hematoma of his left thigh. Initial investigations did not show any conclusive abnormalities of primary or secondary hemostasis. Subsequent tests demonstrated a type 1 deficiency of antiplasmin. Treatment ...

Alpha II Antiplasmin Deficiency Complicating Pregnancy: A Case Report

Background. Alpha II antiplasmin is a protein involved in the inhibition of fibrinolysis. A deficiency in this protein leads to increased hemorrhage. It is inherited in an autosomal recessive fashion. Case. 30-year-old Gravida 1, Para 0, presented for prenatal care with her first and subsequently her second pregnancy. Her medical history was significant for a known deficiency in alpha II antipl...

A familial hemorrhagic diathesis in a Dutch family: an inherited deficiency of alpha 2-antiplasmin.

This study concerns a case of congenital homozygous deficiency in alpha 2-antiplasmin associated with a severe hemorrhagic diathesis. Heterozygous family members also show a mild bleeding tendency. The propositus is a 17-yr-old male born of white parents and showing a severe hemorrhagic diathesis characterized by spontaneous bleeding in the joints since his early childhood. He was originally su...