Navigating Hemostasis of Bleeding Among Children With β-Thalassemia

Area of Science:

• Hematology
• Pediatric Hematology
• Thrombosis and Hemostasis

Background:

• Beta-thalassemia patients frequently exhibit a bleeding phenotype, but the underlying causes are not fully understood.
• Assessing coagulation and platelet function is crucial for understanding bleeding diathesis in this population.

Purpose of the Study:

• To investigate the coagulation profile and platelet aggregation patterns in children with beta-thalassemia and a history of bleeding.
• To identify potential correlations between bleeding symptoms, transfusion history, age, and chelation therapy.

Main Methods:

• Fifty children with beta-thalassemia and a positive bleeding history were enrolled.
• Evaluated bleeding phenotype through clinical history and examination.
• Performed complete blood count, prothrombin time, international normalized ratio, and platelet aggregometry.

Main Results:

• Mucosal bleeding was observed in 96% of patients.
• A significant proportion of patients showed decreased platelet aggregation in response to ristocetin (68%), adenosine diphosphate (64%), and arachidonic acid (64%).
• Impaired aggregation correlated negatively with blood transfusion frequency and patient age. Chelation therapy (deferiprone) and hydroxyurea were associated with specific platelet function defects.

Conclusions:

• Platelet function defects are a significant factor contributing to the bleeding phenotype in beta-thalassemia children.
• These findings highlight the need to consider platelet dysfunction in the management of bleeding symptoms in beta-thalassemia.
• Further research into the impact of chelation and other therapies on platelet function is warranted.