Glanzmann?s thrombasthenia does not protect from severe coronary disease

MESCHENGIESER SS; SÁNCHEZ LUCEROS A; BERMEJO EI; ALBERTO MF; LAZZARI MA

Kyoto

Congreso; XXIII Congress of the International Society on Thrombosis and Haemostasis; 2011

International Society on Thrombosis and Haemostasis

In Glanzmann thrombasthenia a severe lifelong bleeding tendency is very common but the development of an arterial thrombotic event is unexpected.We describe a female patient with a history of frequent nose bleeding, easy bruising and gingival bleeding diagnosed as Glanzmann thrombasthenia (GT) at the age of twelve. Tooth extractions were performed at our Odontology section with local measures without platelet transfusion. She had three pregnancies. The first vaginal delivery was uneventful; in the second one there was profuse bleeding that required curettage and platelet transfusion and in the third oneplatelets were transfused before delivery. At the age of 35, she presented an anterior acute myocardial infarction.Saphenous vein bypass graft was performed with poor results and closure of the by-pass. Stem cell implantation was proposed. The patient never smoked but she had diabetes and hypercholesterolemia plus oral contraceptives due to menorrhagia. Discussion: This patient with GT presents a life threatening thrombotic complication in spite of having a severe primary hemostatic defect. The patient?s bleeding symptoms were not as severe as expected in GT, perhaps suggesting a prothrombotic condition counterbalancing the hemorrhagic risk. Diabetes, hypercholesterolemia and oral contraceptives contributed to the coronary disease but the adverse evolution is difficult to explain. Venous thrombotic complications have been reported in patients with GT but this is the first case, to our knowledge, of a severe arterial thrombosis in this thrombocytopathy.