Glanzmann`s Thrombosthenia: A rare cause of Recurrent Epistaxis

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Glanzmann`s Thrombosthenia: A rare cause of Recurrent Epistaxis

Abdul Khalid*, Tariq Baqai*, Murtaza Bukhari**
Department of Medicine, *Azad Jammu & Kashmir Medical College, Muzaffarabad, **MBBS Medical College Meerpur, AJK University, Muzaffarabad Azad Kashmir

Abstract

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 re-cruited 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.
Key words: Glanzmann`s thrombasthenia, inherited platelet disorders, epistaxis, platelet function studies. Glycoprotein IIb/IIIa.

         

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