ABSTRACT
Glanzmann thrombasthenia is a rare autosomal recessive disease, secondary to a deficiency in platelet membrane glycoproteins GP IIb IIIa, with a defect of platelet aggregation. The result is a disturbance of the primary haemostasis leading to more or less significant haemorrhages, appearing from childhood. We report a case of a female child, 11 months of age, Born of a first-degree consanguineous marriage, without any haemorrhagic familial history. Who presented a abundance dental bleeding following a dental thrust. The biological assessment carried out in her showed a normal blood count; There is no thrombocytopenia and the platelets have a mean average volume of 9.4 fl. prothrombin time, activated partial thromboplastin time and fibrinogen level are normal, The time of platelet occlusion measured on the PFA-200® automaton is elongated: it is greater than 300 seconds in the presence of collagen/epinephrine and collagen/ADP. The analysis of the Willebrand factor does not reveal any abnormality. On the other hand, the exploration of the platelet functions shows a lack of aggregation whatever the platelet activator used: there is no aggregation in the presence of collagen, ADP, arachidonic acid, Aggregation induced by ristocetin is highly disrupted. Immunophenotyping of platelet glycoproteins and the study of expression of glycoproteins after activation by thrombin by flow cytometry show a lack of expression of Gp IIb IIIa [CD41]. This method made it possible to measure the level of Gp IIb IIIa at 1%. Furthermore, factor VIII [coagulant] is greater than 110% and factor IX [coagulant] is at 115%. The diagnosis of Glanzmann type I disease was made