Glanzmann Thrombasthenia Treatment & Management
- Author: Zonera Ashraf Ali, MBBS; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP more...
Patients with Glanzmann thrombasthenia who are bleeding require platelet transfusion. A hematologist may be consulted for transfusion recommendations.
Because patients are likely to require multiple transfusions during their lifetime, they should receive leukocyte-depleted blood products, in order to avoid platelet alloimmunization. Leukocyte depletion can be accomplished with mechanical filtration. Only filtered blood products should be given. Use of platelets matched via human leukocyte antigen (HLA) is a further attempt to prevent platelet alloimmunization.
Other treatment considerations include the following:
Patients should be vaccinated against hepatitis B due to the infectious risks associated with multiple transfusions
Consider oral contraceptives to control menorrhagia
Recombinant activated factor VII (rFVIIa) has been used in patients with antibodies to platelet glycoprotein IIb/IIIa and/or HLA that render transfusions ineffective. In July 2014, the US Food and Drug Administration (FDA) approved rFVIIa (NovoSeven RT) for treatment of bleeding episodes and perioperative management in adults and children with Glanzmann thrombasthenia that is refractory to platelet transfusions, with or without antibodies to platelets.
Patients with Glanzmann thrombasthenia who require surgical intervention should receive platelet transfusions preoperatively, to prevent excessive bleeding during surgery. Further platelet transfusions are considered, based on maintaining hemostasis. Alternatively, rFVIIa may be useful for preventing or controlling perioperative bleeding.
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