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Case Report Open Access
Background: Fatal bleeding episodes due to spontaneous mucocutaneous bleeding are common in Glanzmann`s Syndrome (GT). Control of such bleeding with imperative approaches remains challenging. The unanticipated rare genetic GT hematological disorder of platelet function is life-threatening particularly from the surgical interventions. It is regularly controlled by medical therapies (including; systemic recombinant factor VIIa, local anti-fibrinolytic agents and blood/platelet transfusions) and surgical therapies (including; electrocautery, laser coagulation, and embolization). Case Description and Management: We describe here, for the first time in country, an uncircumcised 10-years old Saudi boy with GT diagnosed during a regular circumcision surgical intervention for relieving phimosis due to edematous enlarged glans penis with bluish skin coloration. The post circumcision life-threatening bleeding - that necessitated redoing the surgery with deep suturing - was confirmed upon hematological consultation to be a GT bleeding disorder. Repeated transfusion of packed red blood cells reaching 8 units significantly induced effective cessation of bleeding within 24 hours, discharge within 72 and complete wound healing without complications within a week. Conclusions: Although very rare, GT should be anticipated upon surgical intervention with secured packed red blood cells, concentrated platelets and rFVIIa (the latter two were available for our case). This case report represents the first description for successful treatment of life-threatening hemorrhage in a GT patient in Saudi Arabia and molecular investigations are planned since his sisters gave a history of profuse menstruation.
Glanzmann`s syndrome, Bleeding disorder, Platelet function, Profuse menstruation, Medical Case Reports