Definition:
Glanzmann's thrombasthenia is an extremely rare disorder of the blood, wherein the platelets lack glycoprotein IIb/IIIa. Hence, no fibrinogen bridging can arise, and bleeding time is significantly prolonged.
Diagnosis:
A hematologist confirms the diagnosis and makes transfusion consultations.
Treatment:
Patients with Glanzmann thrombasthenia who are bleeding need platelet transfusion. Because patients are likely to need multiple transfusions during their lifetime, take care to avoid platelet alloimmunization. Prevention is best accomplished by utilizing leukocyte-depleted blood products. Leukocyte depletion can be done with mechanical filtration. Only filtered blood products should be administered. Use of platelets matched via human leukocyte antigen (HLA) is a further attempt to hinder platelet alloimmunization.
Symptoms and Signs:
Characteristically, there is increased mucosal bleeding. The bleeding tendency is variable but may be extreme. Affected persons present with moderate bleeding and normal platelet morphology. Aggregation of platelets happens in response to ristocetin, but not to other agonists such as ADP, thrombin, collagen or epinephrine.
Causes:
Glanzmann thrombasthenia is a genetic condition, while rare acquired forms caused by antibodies against GP IIb/IIIa have been described.
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