BLEEDING SYNDROM AND ISOLATED ALPHA-2 ANT IPLASMIN DEFICIENCY - A CASE-REPORT

An isolated alpha 2 plasmin inhibitor deficiency is reported in a 33 y ears old male, presenting repeated intramuscular hematomas since 5 yea rs, spontaneously or after minor traumas. None of other family members were suffering from abnormal bleeding. Screening hemostatic examinati ons were normal except for a moderately shorted euglobulin lysis time (2 hours). Evaluation of fibrinolysis parameters (plasminogen, plasmin ogen activator inhibitor type 1, tissue plasminogen activator, fibrin and fibrinogen degradation products, alpha 2 plasmin inhibitor) showed normal values except for alpha 2 plasmin inhibitor which is markedly decreased (activity: 14%, antigen: < 5 %). Familial hemostasis investi gations have not been performed. This isolated alpha 2 plasmin inhibit or deficiency has been confirmed by two repeated control prelevments. Bleedings episodes were treated with antifibrinolytics agents (tranexa mic acid). This case report shows the importance of the diagnostic app roach in the laboratory to detect such rare hemostatic abnormalities a ssociated with bleeding tendency.