Alpha 2-antiplasmin deficiency in a Sudanese child: a case report

The plasma serine protease inhibitor alpha 2-antiplasmin (α₂-AP, otherwise known as α₂-plasmin inhibitor) is a rapid-acting plasmin inhibitor recently found in human plasma, which seems to have a significant role in the regulation of in vivo fibrinolysis. Congenital deficiency of α₂-AP is extremely uncommon.

We report here a case of absolute deficiency of α₂-AP in an 11-year-old Sudanese boy, who had a lifelong intermittent hemorrhagic tendency (gum bleeding, epistaxis, and exaggerated bleeding after trauma). Coagulation tests including prothrombin time, partial thromboplastin time, thrombin time, bleeding time, platelet count, clot retraction test, antithrombin, and factor VIII levels were within normal limits. Hepatic function tests and complete blood count were also normal. The main interesting finding in this patient was that the whole blood clot lysis was extremely fast, completed within 5-8 hours. The second abnormal finding is that the euglobulin clot lysis time was short. Nevertheless, the concentration of α₂-AP in the patient's plasma was 0.2 IU/ml (reference range is 0.80-1.20 IU/ml). The addition of pooled plasma (with normal α₂-AP) to the patient's whole blood corrected the accelerated fibrinolysis.

The study showed that α₂-AP deficiency resulted in uninhibited fibrinolysis that caused the hemorrhagic tendency in this patient. Thus, this report demonstrates the significant role of α₂-AP in coagulation.