This protocol addresses patients with Glanzmann's thrombasthenia who present with severe or life-threatening bleeding — or require major surgical intervention — and in whom the preceding line of hemostatic therapy did not achieve adequate hemostasis.
The prior approach involved platelet transfusion in combination with rFVIIa and/or antifibrinolytics (tranexamic acid) for severe or life-threatening bleeding and major surgical procedures. Escalation to the current protocol is indicated when that regimen fails to achieve hemostasis.
For patients with severe recurrent bleeding who have not responded to prior hemostatic measures, an allogeneic transplantation-based strategy is among the approaches described in the evidence base — but the full protocol specifies the conditioning framework and eligibility criteria.
Allogeneic stem cell transplantation has been successfully performed in selected patients with severe recurrent bleeding using reduced intensity conditioning with good clinical outcomes.
DOI: 10.3324/haematol.2018.214239
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