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June 12, 2017Expert Opinion on Orphan Drugs

Glanzmann’s thrombasthenia: strategies for identification and management

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Key result

Recombinant factor VIIa is recommended over platelet transfusion in Glanzmann's thrombasthenia to prevent anti-αIIbβ3 development.

Population

Patients with Glanzmann's thrombasthenia (GT), a rare inherited platelet disorder

Design

Review

Authors

MPMan‐Chiu PoonGMGiovanni Di MinnoRZRainer B. Zotz

Discussion

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Member takes

Overview

May guide rFVIIa use in refractory or antibody-positive GT; leaves open prospective trials before practice change.

Structured PICO

P
Population
Patients with Glanzmann's thrombasthenia (GT), a rare inherited platelet disorder
I
Intervention
Management strategies including recombinant factor VIIa (rFVIIa) and platelet transfusion
O
Outcome
Bleeding management and prevention of anti-αIIbβ3 development

rFVIIa is recommended over platelet transfusion in specific Glanzmann's thrombasthenia patients to manage bleeding and prevent alloimmunization, particularly in women of reproductive age.

Cite This Study

Poon et al. (2017) conducted a review in Glanzmann's thrombasthenia. Recombinant factor VIIa (rFVIIa) vs. Platelet transfusion was evaluated. Recombinant factor VIIa is recommended over platelet transfusion in Glanzmann's thrombasthenia patients with platelet antibodies, refractoriness, or type I GT to prevent anti-αIIbβ3 development.

synapsesocial.com/papers/6aa607cb39a5015a928e4858https://doi.org/10.1080/21678707.2017.1341306
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Therapeutic expression of the platelet-specific integrin,  IIb 3, in a murine model for Glanzmann thrombasthenia2005 · 90 citations
  2. 2Recombinant factor VIIa enhances deposition of platelets with congenital or acquired αIIbβ3 deficiency to endothelial cell matrix and collagen under conditions of flow via tissue factor–independent thrombin generation2003 · 105 citations
  3. 3A study protocol for a randomised controlled trial evaluating clinical effects of platelet transfusion products: the Pathogen Reduction Evaluation and Predictive Analytical Rating Score (PREPAReS) trial2016 · 30 citations
  4. 4Demonstration of novel gain‐of‐function mutations of αIIbβ3: association with macrothrombocytopenia and glanzmann thrombasthenia‐like phenotype2013 · 59 citations
  5. 5Clinical Practice Guidelines From the AABB2016 · 1,129 citations