Glanzmann's Thrombasthenia Complicating Pregnancy.

Abstract

Glanzmann’s thrombasthenia is a rare autosomal recessive hemorrhagic disorder caused by abnormal platelet glycoprotein complex (GP IIb-IIIa) presenting with hemorrhagic symptoms. Pregnancy is not uncommon because fertility is not affected but an association is rare. Also delivery often results in haemorrhage. Newborn thrombocytopenia is occasionally severe, but is always transitory. We report a 21-year old primigravida, who underwent vaginal delivery at term with IUGR and Oligohydramnios. Glannzmann’s thrombasthenia was characterized by aggregation and this patient went undiagnosed till adolescence until evaluated for puberty menorrhagia which is uncommon. She received single donor platelet transfusion during the intrapartum period along with tranexemic acid. Platelet transfusion may result in alloimmunization and make successive transfusions less effective. We did not use Factor VII in the intrapartum period due to cost and suspected fetal effects. Postnatally she had hematuria settled on the 2nd day with conservative management. We present this case because of association of Glanzmann’s thrombasthenia with IUGR and oligoamnios the only reported case so far.