Alpha 2-Antiplasmin Deficiency
Patients with homozygous alpha 2-antiplasmin deficiency can present early in childhood with bleeding from the umbilical cord and have a lifelong bleeding tendency.7
The bleeding manifestations resemble those of a patient with congenital hemophilia and may include the following:
- Delayed onset of bleeding after minor trauma
- Prolonged bleeding from cuts and wounds, including mucosal bleeding
- Increased bruising and hematomas, including muscle bleeding
- Bleeding into joints after trauma rather than spontaneous joint bleeding
- Excessive postsurgical bleeding (may be a clue in milder cases)
- Excessive bruising
- Increased bleeding after use of nonsteroidal anti-inflammatory drugs (NSAIDs)
Prolonged bleeding has been reported after tonsillectomy, adenoidectomy and dental extraction. Spontaneous hemothorax, hematuria, epistaxis and muscle hematomas also have been described in affected individuals. Many patients with unusual intramedullary hemorrhage into the diaphysis of long bones have been described, occurring either spontaneously or with trauma.10
Heterozygous alpha 2-antiplasmin deficiency may be associated with milder bleeding episodes and most patients may be asymptomatic. Prolonged bleeding may occur following dental or surgical procedures. Bleeding symptoms may sometimes appear with advancing age, as a result of falling levels of alpha 2-antiplasmin.11
Coexisting common disorders like von Willebrand disease or Factor V Leiden may modify the bleeding phenotype and account for the variable clinical expression seen.
Bleeding with miscarriage, placental abruption and postpartum hemorrhage in women following caesarean delivery and during pregnancy have been described.10,12 Menorrhagia has been reported too.
Individuals with alpha 2-antiplasmin deficiency experience bleeding episodes because of the premature dissolution of the hemostatic plug before repair of tissues and vessels. Bleeding may be delayed after trauma or invasive procedures. Individuals who have severe liver disease and decreased plasma levels may experience acquired deficiency of alpha 2-antiplasmin.7 Decreased levels of alpha 2-antiplasmin have also been reported in patients with renal disease, amyloidosis and disseminated intravascular coagulation, as well as in patients undergoing thrombolytic therapy.13