[Glanzmann's thrombasthenia: first case descriptions in sub-saharan africa]

ABSTRACT

Constitutive hemorrhagic diseases that affect primary haemostasis are reportedly rare in sub-Saharan Africa This study arrived to report within a Congolese family live cases of Glanzmann's thromboasthenia 5 cases of the congenital form of Glanzmann's thromboasthenia were depictecd in a Congolese family. The disease was first discovered with a young student who was transferred in France who had shown a tendency to develop hemorrhages since childhood. This tendency was enhanced following abdominal surgery to treat peritonitis. Like the other 3 cases, she had a prolonged bleeding time, albeit with normal von Willebrand factor plasma values. A 7 year old girl died following appendectomy from post-surgery hemorrhages. In this young patient, platelet aggregation could be induced only by ristocetine all other conventional agonists failed. Flow cytometric analysis showed the total absence of GPIIbIIIa. The hemorrhages in the girls could be managed by cyclic administration of oestrogens and iron supplementation .Serological analysis showed this patient to be Positive for hepatitis C virus antibodies. This first description of Glanzmann's thromobo - asthenia in Blacks in sub-Saharan Africa shows the necessity of establish inter-hospital cooperationfor the improvement of the management of constitutive hemorrhagic diseases in the Hematology words