A case of congenital prothrombin deficiency and idiopathic thrombocytopenic purpura in a pregnant female

Abstract

Hereditary prothrombin deficiency is an autosomal recessive disorder with an estimated incidence of 1 in 2 million . Presentation of the disease is variable; however, it is usually associated with moderate to severe bleeding tendencies including muscle hematomas, hemarthrosis, intracranial, mucosal, and postoperative bleeding. Here we report a case of a 35-year-old pregnant woman with congenital hypoprothrombinemia and idiopathic thrombocytopenic purpura, review the literature, and discuss its epidemiology, presentation, diagnosis, and treatment.

Management of rare inherited bleeding disorders: Proposals of the French Reference Centre on Haemophilia and Rare Coagulation Disorders

“…On the basis of two retrospective studies on 22 women, one injection of 20–40 IU/kg of PCC should be considered, from the beginning of labour, to prevent bleeding (Grade 2C) 17 (Table 5).…”