Abstract
Patients with inherited bleeding disorders are rare in obstetric practice but present with prolonged bleeding even after minor invasive procedures. They require a combined approach with obstetric and hematological management of each case, including the neonatal management of a possibly affected fetus. We present the case of a pregnancy in a patient with combined Factor VII deficiency and Glanzmann's thrombasthenia, the successful obstetric and hematological management of the case, and a review of the literature.
Highlights
Obstetric protocols for the management of patients with inherited bleeding disorders vary regarding the possible need for systemic treatment of haemostatic support, for example, factor concentrates, plasma, and desmopressin, during treatment of patients with inherited bleeding disorders
At the end of pregnancy, the prothrombin time of the patient was 46% and the bleeding time was 9 minutes, and FVII levels had increased to 9% but platelet aggregation studies and expression of GPIIb-IIIa were not repeated
Factor VII deficiency should be suspected in patients with a prolonged prothrombin time and normal thromboplastin time and the diagnosis confirmed with the Factor VII specific assay
Summary
Obstetric protocols for the management of patients with inherited bleeding disorders vary regarding the possible need for systemic treatment of haemostatic support, for example, factor concentrates, plasma, and desmopressin, during treatment of patients with inherited bleeding disorders. In patients with an inherited bleeding disorder, relatively minor invasive procedures may precipitate prolonged bleeding. This excessive bleeding is distressing for both patients and clinicians and can delay completion of the procedure, compromise wound healing, and predispose to infection. We present the case of a 21-year-old pregnant woman with congenital deficiency of Factor VII and Glanzmann’s thrombasthenia, a platelet dysfunction disease. Patients with Factor VII deficiency are often asymptomatic but may bleed during invasive procedures. Glanzmann’s thrombasthenia was first identified in 1918 [4] and described as an inherited disorder characterized by platelets of normal size that failed to spread on a surface and failed to support clot retraction [5]. Heterozygotes with half the normal concentration of platelet GPIIb-IIIa have no abnormalities of platelet function and no clinically significant bleeding [8]
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