Glanzmann’s Thrombasthenia: A Rare Cause of Recurrent Profuse Epistaxis

Abstract

Though epistaxis is a commonplace emergency encountered in the Otorhinolaryngology clinic, recurrent, severe and intractable cases are relatively less common. In those cases where no local causes are found that could explain such episodes, systemic causes, including hematological disorders should be considered, and thoroughly explored. However, routine hematological investigations often fail to identify a definite cause. Here we report about a 23 year-old woman presenting with severe recurrent epistaxis due to Glanzmann's thrombasthenia-a rare, autosomal recessive disease. Never before Glanzmann's thrombasthenia has been reported to present with severe epistaxis as its sole feature.

Glanzmann's disease; gingival bleeding despite good OH

“…This glycoprotein is a receptor for fibrinogen, which explains why this abnormality frustrates the molecular bridging between platelets. Excessive bleeding after dental extractions can be the first manifestation but GT has also presented with petechiae, ecchymoses, menorrhagia or epistaxes – any of which can be the sole clinical manifestation . Rarely, GT causes haemarthroses, gastrointestinal bleeding or haematuria.…”

Lethal Epistaxis

“…Thrombocytopenia may be idiopathic or associated with a wide variety of underlying conditions including infectious agents such as Epstein–Barr virus and tuberculosis . Rarer disorders such as Glanzmann thrombasthenia, an autosomal recessive disorder of platelet aggregation, may not present until early adult life when it may manifest with unexpected profuse epistaxis . Epistaxis was a presenting feature in 34% of children with aplastic anemia in one study from Pakistan .…”