Rétinopathie drépanocytaire proliférante révélatrice d'une thrombasthénie de Glanzmann

Glanzmann thrombasthenia is a rare qualitative platelet abnormality of autosomal recessive transmission caused by absence of GP IIb/IIIa and resulting in disturbance of platelet aggregation and bleeding time extension. We report the case of a 16-year-old female suffering from SC sickle cell disease and presenting with bilateral vitreous hemorrhage revealing Glanzmann thrombasthenia. Despite vitrectomy, the functional prognosis was poor. Indeed, association of a severe hemorrhagic disease and sickle cell retinopathy makes surgical management difficult.