[Hemorrhagic syndrome and isolated alpha 2-antiplasmin deficiency. Apropos of a case].
An isolated alpha 2 plasmin inhibitor deficiency is reported in a 33 years old male, presenting repeated intramuscular hematomas since 5 years, spontaneously or after minor traumas. None of other family members were suffering from abnormal bleeding. Screening hemostatic examinations were normal except for a moderately shorted euglobulin lysis time (2 hours). Evaluation of fibrinolysis parameters (plasminogen, plasminogen 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 investigations have not been performed. This isolated alpha 2 plasmin inhibitor deficiency has been confirmed by two repeated control prelevments. Bleedings episodes were treated with antifibrinolytics agents (tranexamic acid). This case report shows the importance of the diagnostic approach in the laboratory to detect such rare hemostatic abnormalities associated with bleeding tendency.
Guermazi
S
S
Laboratoire d'Hématologie, Institut Pasteur de Tunis, Tunis-Belvedere, (Tunisie).
Khelif
A
A
Conrad
J
J
Ennabli
S
S
Dellagi
K
K
D000328:Adult; D005342:Fibrinolysis; D006470:Hemorrhage / Q000209:etiology*; D006801:Humans; D008297:Male; D000979:alpha-2-Antiplasmin / Q000172:deficiency*
D000979:alpha-2-Antiplasmin