Treatment and outcomes for epistaxis in children with Glanzmann's thrombasthenia

Abstract

To date, no form of medical or surgical intervention in children with this condition has consistently demonstrated its ability resolve nasal hemorrhage. The role of the otolaryngologist is to control bleeding during major episodes of nasal hemorrhage that do not respond to medical management. These patients usually have remarkable improvement in the frequency and severity of epistaxis in adolescence and then require much less aggressive therapy.

Glanzmann’s thrombasthenia – newer treatment options

“…For mild-to-moderate bleeding in GT, the following approach is generally practiced: i) Local pressure/use of fibrin glue/human thrombin or use of other products such as collagen matrix may be effective in cases of mild bleeding [19,20]. Gelatin sponges soaked with antifibrinolytics such as epsilon aminocaproic acid (EACA) or tranexamic acid is often used locally to stop superficial bleeding.…”

What To Do When You Suspect an Inherited Platelet Disorder

“…Topical sealants can also be used to manage gum bleeding and epistaxis. 52 Conservative management with topical sealants and/or pressure is often sufficient. Cauterization may be effective in short-term management, but may result in more frequent and recalcitrant nosebleeds in the long term and should be avoided if possible.…”

Nasal Packing with Strips of Cured Pork as Treatment for Uncontrollable Epistaxis in a Patient with Glanzmann Thrombasthenia

“…In particular, evidence suggests a quantitative and qualitative abnormality of the IIb/ IIIa integrin fibrogen receptor, a key intercellular communication molecule. [1][2][3][4][5][6] Two types of GT have been identified; the more-severe type I has no glycoprotein IIb/IIIa receptor complex, and the less-severe type II has varied amounts of receptor molecules. Normal-functioning platelets undergo a conformational change and aggregation upon exposure to ade nosine diphosphate, thrombin, and collagen, all of which are known platelet agonists.…”

“…2 Reports have suggested higher incidences among Iranian, Indian, and Arabic populations. 4 Management includes medical and surgical options, all with various degrees of success. In particular, control of nasal hemorrhage has been a frequent and challenging problem for patients with GT.…”

“…Clinical researchers recently reported that approximately 82% of bleeding events in patients with GT involved the nasal cavity (ie, epistaxis). 4 Recent literature reports describe medical therapies that use recombinant factor VII, antifibrinolytic agents, or platelet transfusions (with or without red blood cells) to treat bleeding outbreaks. [4][5][6][7][8][9] Although platelet transfusion is among the first-line therapies for GT, isoimmunization against glycoprotein IIb/IIIa on normal transfused platelets precludes the use of platelet transfusion because of possible future lifethreatening hemorrhage.…”