glanzmann's thrombosthenia is one of the rare inherited platelet disorder charcerized by a deficiency or dysfunction of the glycoprotien IIb/IIIa(integrin aIIbB3)complex on the platelet surface .this defect impairs platelets aggregation,leading to life longtoward mucocutanous bleeding ,epistaxis ,menorrhagia. Management includes preventive and theraputic manners like: platelet transfusion and reFVII with other local treatment . BMT will be another option for complicated cases and gene therapy will be the future option of treatment.
Learning Objectives:
through my presentation I will highlight symptoms and signs and mode of inheritence ,investigation and treatment for Glanzmann's thrombasthenia