Glanzmann thrombasthenia (GT) is a rare and often underdiagnosed congenital bleeding disorder caused by mutations in the genes encoding glycoproteins GPIIb or GPIIIa, resulting in platelet dysfunction. Inherited in an...Glanzmann thrombasthenia (GT) is a rare and often underdiagnosed congenital bleeding disorder caused by mutations in the genes encoding glycoproteins GPIIb or GPIIIa, resulting in platelet dysfunction. Inherited in an autosomal recessive manner, GT is characterized by the inability of platelets to aggregate. Clinically, it presents with mucocutaneous bleeding, such as easy and extensive bruising, severe epistaxis, menorrhagia, gingival bleeding, postpartum hemorrhage, and unexpected bleeding following procedures, despite a normal platelet count. We present a case involving a 6-year-old male patient who experienced spontaneous gingival bleeding for the past 4 weeks due to the eruption of his first permanent molars. The bleeding was particularly severe at night, disrupting the child’s sleep. The patient had been diagnosed with GT at the age of 16 months. Dental management was pursued, and the use of tranexamic acid mouthwash, combined with meticulous oral hygiene, resulted in an excellent response.展开更多
文摘Glanzmann thrombasthenia (GT) is a rare and often underdiagnosed congenital bleeding disorder caused by mutations in the genes encoding glycoproteins GPIIb or GPIIIa, resulting in platelet dysfunction. Inherited in an autosomal recessive manner, GT is characterized by the inability of platelets to aggregate. Clinically, it presents with mucocutaneous bleeding, such as easy and extensive bruising, severe epistaxis, menorrhagia, gingival bleeding, postpartum hemorrhage, and unexpected bleeding following procedures, despite a normal platelet count. We present a case involving a 6-year-old male patient who experienced spontaneous gingival bleeding for the past 4 weeks due to the eruption of his first permanent molars. The bleeding was particularly severe at night, disrupting the child’s sleep. The patient had been diagnosed with GT at the age of 16 months. Dental management was pursued, and the use of tranexamic acid mouthwash, combined with meticulous oral hygiene, resulted in an excellent response.
