Bone marrow transplantation in severe Glanzmann's thrombasthenia with antiplatelet alloimmunization

Glanzmann's thrombasthenia is an autosomal recessive disorder characterized by a lack of platelet aggregation due to the absence of platelet glycoprot ein IIb and IIIa, Usually, the disease leads to mild hemorrhage but sometim es bleeding is severe enough to be life-threatening. We report the case of a 16-year-old girl, presenting with very severe type 1 Glanzmann's thrombas thenia, successfully treated with an HLA-identical sibling bone marrow tran splant (BMT), We also update the clinical and laboratory data of her brothe r, who had received a BMT 16 years ago for the same disease. In the light o f these two cases and two others published in the literature, we discuss th e indications for BMT from HLA-identical sibling donors in Glanzmann's thro mbasthenia, Alloimmunization against the missing platelet GPIIb/IIIa comple x and severity of bleeding episodes may constitute sufficient criteria for allogeneic BMT after careful assessment of the risk-benefit of such a proce dure, although this remains exceptional in this disease.