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[A homozygous quantitative defect of alpha 2-antiplasmin in a family from central Slovakia]
1Department of Hematology, F.D. Roosevelt's Hospital, Banska Bystrica, Slovakia.
Alpha2-antiplasmin is the main inhibitor of plasma fibrinolytic system. An inborn defect of alpha2-antiplasmin was first described by Koie et al. in 1978 in connection with severe haemorrhage syndrome. The authors present a case report of 45 years old woman living in middle Slovakia with severe haemorrhagic syndrome started in childhood (epistaxis, skin and muscle hematomas, appendectomy with 6 weeks recovery, hardly manageable haemorrhage after teeth extractions, menorrhage, metrorrhage). Laboratory tests were negative for platelet function defects and coagulation system defects. Low level of alpha2-antiplasmin activity (10%) was detected with use of synthetic chromogenic substrate method and low amount (2%) with ELISA method. In asymptomatic daughter was decreased level of alpha2-antiplasmin (activity 51%, quantity 32%) detected. On the basis of patient history, laboratory investigations and comparison with published cases the haemorrhagic syndrome is considered to be an inborn homozygous quantitative defect of alpha2-antiplasmin. Detection of the defect in further haemorrhagic and risk situations (including laparotomy, multiple teeth extractions, obstetric surgery) led to following therapeutic measures: careful local care [by procedures], tranexamic acid in sufficient dose [4 g/day in continual i.v. infusion, or 4 x 1 g in 1/hour infusions, 4 x 1 g perorally], in sufficient duration [14 days by procedures]. Therapeutic approach after detection of the defect is efficient. (Tab. 2, Ref. 13.)
Alpha2-antiplasmin is the main inhibitor of plasma fibrinolytic system. An inborn defect of alpha2-antiplasmin was first described by Koie et al. in 1978 in connection with severe haemorrhage syndrome. The authors present a case report of 45 years old woman living in middle Slovakia with severe haemorrhagic syndrome started in childhood (epistaxis, skin and muscle hematomas, appendectomy with 6 weeks recovery, hardly manageable haemorrhage after teeth extractions, menorrhage, metrorrhage). Laboratory tests were negative for platelet function defects and coagulation system defects. Low level of alpha2-antiplasmin activity (10%) was detected with use of synthetic chromogenic substrate method and low amount (2%) with ELISA method. In asymptomatic daughter was decreased level of alpha2-antiplasmin (activity 51%, quantity 32%) detected. On the basis of patient history, laboratory investigations and comparison with published cases the haemorrhagic syndrome is considered to be an inborn homozygous quantitative defect of alpha2-antiplasmin. Detection of the defect in further haemorrhagic and risk situations (including laparotomy, multiple teeth extractions, obstetric surgery) led to following therapeutic measures: careful local care [by procedures], tranexamic acid in sufficient dose [4 g/day in continual i.v. infusion, or 4 x 1 g in 1/hour infusions, 4 x 1 g perorally], in sufficient duration [14 days by procedures]. Therapeutic approach after detection of the defect is efficient. (Tab. 2, Ref. 13.)