α₂-Antiplasmin: Functional Characterization and Metabolism in a Heterozygote Deficient Patient

Summary

An 81-year-old male with a mild life-long bleeding history and an α₂-antiplasmin (α₂-AP) plasma level of 55% biological activity and 41% antigen activity (normal range 80-140%) was studied. The ratio of plasminogen binding (PB): non-plasminogen binding (NPB) α₂-AP assayed by modified crossed immunoelectrophoresis (CIE) was 7.3/2.7 (controls 6.3 ± 0.49 SD/3.7 ± 0.49 SD). The patient’s α₂-AP showed decreased affinity for fibrin, i. e. 8.3% versus 32.4% of normal control α₂-AP associated with fibrin during clotting of plasma. A metabolic study performed with human purified ¹²⁵I-α₂-AP(PB/NPB 7.7/2.3) showed a plasma radioactivity disappearance half-life of 72.9 h (n 60.1 ± 5.3 h) with a normal fractional catabolic rate and a reduced absolute catabolic (synthetic) rate of 0.70 mg/kg/day (n 2.10 ± 0.60 mg/kg/day). The exchange between the central and third compartment was increased. The increased α₂-AP PB form and the increased plasma radioactivity disappearance half-life are suggestive of a slower conversion of the PB form into the NPB form and/or slower degradation of the PB form. The bleeding tendency in this patient could be explained by decreased synthesis of α₂-AP and decreased binding to fibrin.

Keywords

α₂-Antiplasmin metabolism - Characterization - Heterozygote deficiency

• References

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