Glanzmann Thrombasthenia: A Case Report in Moroccan Child

摘要

Glanzmann thrombasthenia is a hereditary hemorrhagic problem and is characterized by a qualitative or quantitative deficiency of the platelet glycoprotein GPIIb-IIIa, responsible for the reduction of platelet aggregation. The reported case concerns an 11-year-old child admitted to the Clinical Hematology Department of the University Hospital Mohamed 6 for epistaxis and gingivorrhagia. The questioning of the father revealed the concept of consanguinity, as well as his history of death and his hemorrhagic syndrome. The biological assessment performed in a patient shows a normal blood count; it is not particularly thrombocytopenia and the platelets have an average volume of 10.6 fL, the prothrombin time (PT), the partial thromboplastin time with activator (APTT) are normal. The platelet occlusion time (POT) is lengthened. The von Willebrand factor has not been investigated: the antigen (VWF-Ag) is 148% and the cofactor of ristocetin (VWF-RCo) is 116%. On the other hand, the exploration of platelet functions shows a lack of aggregation or of the platelet activator used: there is no aggregation in the presence of collagen, ADP, acid arachidonic, and aggregation induced by ristocetin is very disturbed. Immunophenotyping of platelet glycoproteins and the study of glycoprotein expression after thrombin activation by flow cytometry resulting from GpIIbIIIa (CD41), GpIIIa (CD61). The diagnosis is available for this patient is Glanzmann trombasthenia.