[Pregnancy and labor in a primigravida with Glanzmann's thrombasthenia]

Abstract

Glanzmann's thrombasthenia is an autosomal, recessive, inherited haemorrhagic disorder due to the lack of the glycoprotein IIb/IIIa (GP IIb/IIIa) complex of the platelet membrane. The disease is characterized by a prolonged bleeding time and by a severe haemorrhagic mucocutaneous diathesis. The only possible antihaemorrhagic therapeutic measure is the infusion of platelet concentrates, but this is almost constantly associated with the production of antibodies against the GP IIb/IIIa complex that lead to the rapid removal of the infused platelets from the circulation. For this reason, platelet transfusions are usually without efficacy and must be limited to severe, profuse haemorrhages. We report the case of a primigravidae with Glanzmann's thrombasthenia in which a spontaneous delivery was made possible by a series of measures taken during the parturition to avoid haemorrhagic complications, without the use of platelet transfusions.