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A new congenital dysprothrombinaemia is described in a newborn baby girl who presented severe bleeding from the second day of life. Routine coagulation tests showed very prolonged prothrombin time and activated partial thromboplastin time with about 2% prothrombin activity in a one-stage assay. Staphylocoagulase and Echis carinatus venom prothrombin assays were respectively 35% and 25%. The prothrombin antigen level was 47% and its migration in crossed immuno-electrophoresis was abnormal. Family study revealed the presence of both normal and abnormal prothrombin in the plasma of three family members: the father, the mother and the brother. Thrombin generation in a system free from natural inhibitors showed that the abnormal prothrombin was slowly and incompletely activated. The propositus is thought to be homozygous for a 'lazy' dysprothrombin.
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