Guermazi S, Khelif A, Conrad J, Ennabli S, Dellagi K
Laboratoire d'Hématologie, Institut Pasteur de Tunis, Tunis-Belvedere, (Tunisie).
Pathol Biol (Paris). 1997 Jun;45(6):483-6.
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.
据报道，一名33岁男性患有孤立性α2纤溶酶抑制剂缺乏症，自5年前起反复出现肌内血肿，可自发出现或在轻微创伤后出现。其他家庭成员均无异常出血情况。除优球蛋白溶解时间适度缩短（2小时）外，筛查性止血检查均正常。对纤溶参数（纤溶酶原、1型纤溶酶原激活物抑制剂、组织纤溶酶原激活物、纤维蛋白和纤维蛋白原降解产物、α2纤溶酶抑制剂）的评估显示，除α2纤溶酶抑制剂明显降低（活性：14%，抗原：<5%）外，其他参数均正常。未进行家族性止血调查。两次重复的对照检测均证实了这种孤立性α2纤溶酶抑制剂缺乏症。出血发作采用抗纤溶药物（氨甲环酸）治疗。本病例报告显示了实验室诊断方法对于检测此类与出血倾向相关的罕见止血异常的重要性。
