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European Management of Glanzmann's Thrombasthenia: A Survey of Current Clinical Practice
Mathieu Fiore1,2,3, Andrea Artoni4, Robert Klamroth5
1Department of Haematology, University Hospital of Bordeaux, Pessac, France.
Insights
Glanzmann
Area of Science:
- Hematology
- Platelet Disorders
- Rare Diseases
Background:
- Glanzmann's thrombasthenia (GT) is a rare inherited platelet disorder affecting platelet aggregation.
- Diagnosis typically occurs in early childhood, requiring multifaceted management for bleeding.
- A lack of European consensus currently exists for GT management.
Purpose of the Study:
- To understand current clinical management practices for Glanzmann's thrombasthenia across Europe.
- To identify areas for aligning best practices and improving patient outcomes in GT care.
Main Methods:
- An online survey of 57 questions was administered to European hematologists managing GT patients.
- Survey topics included diagnosis, treatment, immunization, peri-operative care, and second-line therapies.
- The survey was conducted by the EAHAD Glanzmann Working Group.
Main Results:
- Physician consensus was found on peri-operative treatment, recombinant factor VIIa use, and antibody development concerns.
- Varied responses were noted regarding antibody screening (≤53% in most countries), HLA-matched platelet access (limited for 55%), and transfusion duration.
- Significant variation exists in diagnostic and therapeutic approaches across European countries.
Conclusions:
- Establishing comprehensive Glanzmann's thrombasthenia management guidelines is crucial.
- Standardized care will enhance patient outcomes and ensure high-quality, effective treatment.
- Guidelines will help standardize care across diverse European healthcare settings.
Introduction:
Glanzmann's thrombasthenia is a rare inherited platelet disorder characterized by a lack of platelet aggregation. Patients tend to be diagnosed in early childhood with treatment strategies involving a multifaceted approach to prevent and manage bleeding episodes. Unfortunately, there is currently no European consensus regarding the management of GT.
Aim:
This initiative aimed to gain an understanding of current clinical management of GT across Europe, with the aim of aligning best practice and improving patient outcomes.
Methods:
The authors, on behalf of the EAHAD Glanzmann Working Group, administered an online survey of 57 questions to European haematologists currently involved in the management of patients with GT. The survey covered topics related to diagnosis, treatment access and selection, immunization, peri-operative management and use of second-line therapies.
Results:
Responses reflected physician consensus around some topics, including peri-operative treatment, use of recombinant factor VIIa, and concerns around antibody development. However, more varied responses were received on topics such as antibody screening (anti-αIIbβ3 antibodies screening conducted by ≤53% of respondents in all countries of interest except France), access to HLA-matched platelet concentrates (none or limited for 55% of respondents) and duration of platelet transfusions for major surgery (13%-31% for 1, 2, 3 and 4 or more days of transfusions).
Conclusion:
Establishing comprehensive guidelines to manage GT will enhance patient outcomes by ensuring patients receive high-quality and effective care as well as standardize care across different healthcare settings.
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