Pregnancy in mother with Glanzmann's thrombasthenia and isoantibody against GPIIb-IIIa: Is there a foetal risk?

Glanzmann's thrombasthenia (GT) is a rare autosomal recessive platelet disorder caused by qualitative or quantitative abnormalities of a platelet glycoprotein complex (GPIIb-IIIa) leading to excessive bleeding. Platelet transfusions are the first-line therapy for severe or persistent bleeding and surgery. Isoantibody against GPIIb-IIIa complexes present on normal platelets can be observed in Glanzmann's thrombasthenia type I patients after platelet transfusion possibly leading to platelet transfusion refractoriness. Pregnancy in Glanzmann's thrombasthenia type I women is rare, and severe bleeding can be observed in the peripartum or late postpartum period. Moreover, pregnancy can contribute to the maternal isoimmunization by the passage of the foetal cells into the maternal circulation. The transplacental passage of the maternal isoantibodies can induce moderate to severe foetal thrombocytopenia. We discuss here the case of in utero death at 31 weeks of gestation due to intracranial haemorrhage in an immunized mother and review the literature. Presence of isoantibody prior to gestation or detected during the index pregnancy must be taken into account in evaluating risk for the mother and the foetus.