SSC Subcommittee on Platelet Physiology

Arterial thrombosis (acute coronary syndrome or stroke) in Glanzmann Thrombasthenia (GT) patients poses a major clinical challenge. These rare but serious events are extremely difficult to manage, given the inherent bleeding risk in GT and the absence of evidence-based therapeutic guidelines. Available data are limited to a few case reports, and the heterogeneity of hemostatic strategies highlights the urgent need for standardized recommendations.

To address this gap, the GlanzArter project aims to retrospectively analyze an international cohort of adult GT patients who have experienced an arterial thrombotic event, including acute coronary syndrome or stroke. The study will describe patients' demographic and biological characteristics, cardiovascular risk factors, diagnostic procedures, hemostatic and interventional management, and outcomes, including bleeding complications. This is an official collaborative project of the ISTH SSC Subcommittee on Platelet Physiology.

The ultimate goal is to identify critical patterns and management strategies that may inform future guidelines for the care of GT patients facing arterial thrombotic events.

We are conducting a retrospective data collection through two structured CRFs (one for acute coronary syndrome, one for stroke). These have been developed by an expert panel in hemostasis, cardiology, and neurology. All data will be pseudonymized and stored in a secured database hosted by Marseille University Hospital (France).

We are now inviting all centers involved in the care of GT patients worldwide to contribute by reporting any case of stroke, transient ischemic attack, acute coronary syndrome, or unstable angina in adult GT patients. Even a single case per center is highly valuable due to the rarity of the condition.

Your participation is essential to help improve the care of these vulnerable patients.

To receive the protocol and CRFs, as well as further information please contact Prof. Paul Saultier (paul.saultier@ap-hm.fr). (See full project description under "Current Projects" on the ISTH website.)

Gastrointestinal bleeding (GIB) is a potentially dreadful bleeding complication in Glanzmann Thrombasthenia (GT) because it is usually diagnosed late, when a significant amount of blood has already been lost, it is challenging to treat and leads to frequent hospitalizations, need of blood- and platelet-transfusions and death. Several cases of severe GIB in patients with GT can be found in the literature. One common cause of occult GIB is gastrointestinal angiodysplasia (GIA), a digestive tract vascular malformation characterized by localized ectasia and thinning of mucosal vessels, linked to dysregulated neoangiogenesis.

However, a systematic evaluation specifically of the frequency and characteristics of GIB in GT, and its possible association with GIA, has never been carried out.

Aim of this study is to assess the prevalence and severity of GIB, its association with GIA and the therapeutic approach adopted for the treatment of GIB, in a large population of Glanzmann Thrombasthenia patients.

We are now performing a retrospective survey asking to all centers involved in the management of GT patients worldwide to contribute to this study by filling in an easy and short questionnaire on GIB in GT.

This is an official initiative of the EHA-SWG on Thrombocytopenia and Platelet Function Disorders in conjunction with the SSC Platelet Physiology of the ISTH (see full project description at the link under Current Projects).

To receive the protocol and CRF and for further information please contact Prof Paolo Gresele (paolo.gresele@unipg.it).