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Coagulation contact phase factors and inhibitors in beta-thalassemia major children
F Schettini1, D De Mattia, G Arcamone
1Institute of Clinical and Preventive Pediatrics, University of Bari, Italy.
Insights
Beta-thalassemia major alters blood clotting in children. Studies show changes in platelet counts, protein levels, and clotting times, impacting hemostasis.
Area of Science:
- Hematology
- Pediatric Medicine
- Thrombosis Research
Background:
- Beta-thalassemia major is a severe inherited blood disorder.
- Hemostasis, the process of stopping bleeding, can be complex in chronic diseases.
- Understanding coagulation disorders in pediatric patients is crucial for management.
Purpose of the Study:
- To investigate hemostatic system alterations in children with beta-thalassemia major.
- To compare coagulation parameters between splenectomized and non-splenectomized patients.
- To identify specific hemostatic markers affected by beta-thalassemia major.
Main Methods:
- Analysis of plasma prekallikrein levels.
- Measurement of platelet count and in vitro platelet aggregability.
- Assay of Protein C, fibrinogen, and vitamin K-dependent proteins.
- Evaluation of activated partial thromboplastin time and normotest.
Main Results:
- Reduced plasma prekallikrein in all patients.
- Increased platelet count and aggregability in splenectomized patients.
- Prolonged activated partial thromboplastin time and reduced normotest.
- Decreased fibrinogen and Protein C in non-splenectomized patients.
Conclusions:
- The hemostatic system is significantly altered in children with beta-thalassemia major.
- Splenectomy influences specific hemostatic parameters, including platelet function.
- Further research is needed to correlate laboratory findings with clinical outcomes.
Abstract:
Selected hemostatic parameters of 23 children affected by beta-thalassemia major were studied and compared to an age- and sex-matched group. Plasma prekallikrein level was reduced in all patients, splenectomized or not. In splenectomized patients, platelet count and in vitro platelet aggregability were significantly increased and Protein C was slightly increased. The activated partial thromboplastin time was prolonged and the normotest reduced. Finally, a reduction in the plasma levels of fibrinogen and of vitamin K-dependent proteins, including the antithrombotic Protein C, was observed in nonsplenectomized patients. Our data indicate that the hemostatic system in patients with thalassemia major may be altered. The relationship between these laboratory changes and clinical manifestations remains to be established.