Alpha 2-Antiplasmin Deficiency
Although congenital deficiency of alpha 2-antiplasmin is a rare disorder, it is important to rule out this disease when evaluating patients with an unknown bleeding disorder. Some of the manifestations of alpha 2-antiplasmin are also associated with other conditions, as follows:
1. Bleeding in the bones similar to the intramedullary hematomas of alpha 2-antiplasmin deficiency has also been described in afibrinogenemia.
2. Other disorders resulting in premature fibrinolysis like Plasminogen Activator Inhibitor-1 (PAI-1) deficiency.
3. Due to alpha 2-antiplasmin’s strong interaction with FXIII, many presenting symptoms are similar to those of FXIII deficiency.
4. Acquired alpha 2-antiplasmin deficiency which may be seen with liver disease (decreased synthesis), disseminated intravascular coagulation (increased consumption), and nephrotic syndrome (urinary loss) or during thrombolytic therapy.Next