Glanzmann`s Thrombosthenia: A rare cause of Recurrent Epistaxis
Keywords:
Glanzmann`s thrombasthenia, inherited platelet disorders, epistaxis, platelet function studies, Glycoprotein IIb/IIIaAbstract
Platelets play an important role in the process of homeostasis in endothelial injuries. Glanzmann`s
thrombasthenia is very rare inherited functional disorder of platelets. It is characterized by failure of
platelet aggregation due to the lack of cell membrane glycoproteins IIb/ IIIa. It presents with bleeding
in young age. The bleeding episodes are usually mild and recurrent but sometimes bleeding may be
severe enough to be life threatening. The platelets are the first major component of coagulation system
at the site of injury and bleeding. In case of trauma platelets adhere to the exposed sub-endothelial tissue.
Platelets release biochemical mediators (Adenosine biphosphate and serotonin) and more cells are
recruited towards the injured area in a process called activation of platelets. We report a twelve years
old boy with recurrent epistaxis where nasal packing was ineffective to control bleeding. His bleeding
profile showed significantly prolonged bleeding time and platelet function studies confirmed the diagnosis.
He was managed with platelet transfusions
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Copyright (c) 2018 Abdul Khalid, Tariq Baqai, Murtaza Bukhari
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