Thrombophilia In Beta Thalassemia

Study Overview
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Brief Summary
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β-thalassemia disease is one of the most common congenital hemolytic anemia commonly found in the malarial belt areas including the Mediterranean, the Middle East, Africa, Southeast Asian countries, and China.

Detailed Description
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β-thalassemia represents a major public health problem in Egypt, it is estimated that there are 1000/1.5 million per year live births born with β-thalassemia. The average life expectancy of patients with β-thalassemia has improved over the last few years as compared to that of in the previous millennium. This has led to the discovery of new set of problems such as increased hypercoagulable state in β-thalassemia like micro infarcts in spleen and lung indicating an activated coagulation pathway. The, incidence of thromboembolism in patients with thalassemia disease is approximately 10 times higher than normal population, it accounts between 1.7 and 9.2%. On the other hand, a study conducted by Chaudhary and Ahmad, 2012 showed decreased aggregation in majority of β-thalassemia patients. Another study conducted by Ibrahim, 1999 had noticed few patients to have bleeding manifestations in the form of epistaxis. Mussumeci et al., 1987 noted that both thrombophilic and anti-thrombophilic proteins were reduced as a consequence of liver damage. The net clinical outcome depends on the fine balance between the prothrombotic and antithrombotic pathways.

Official Title
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Thrombophilia Versus Platelet Dysfunction In Beta Thalassemia

Conditions
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Beta-Thalassemia

Intervention / Treatment
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* Diagnostic Test: PT
* Diagnostic Test: Protein C
* Diagnostic Test: Platelet aggregation by ADP and arachidonic acid


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: All blood samples from thalassemia patients before blood transfusion. In splenectomized patients taking Aspirin, the tests will be performed 7 days after discontinuation of the drug. Exclusion Criteria: Patients with other hemoglobinopathies other than beta-thalassemia. Patients suffering from hepatic or cardiac dysfunctions of another aetiology. Patients with history of familial thrombophilia or use of anticoagulant therapy.

Ages Eligible for Study
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Minimum Age: 4 Years
Maximum Age: 20 Years

Sexes Eligible for Study
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All

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| study group<br>Diagnosed beta-thalassemia patients at Assiut University Hospital. | Diagnostic Test: PT<br>* measuring PT drawn on citrated blood sample<br>Diagnostic Test: Protein C<br>* measuring protein C drawn on citrated blood sample<br>Diagnostic Test: Platelet aggregation by ADP and arachidonic acid<br>* measuring platelet aggregation drawn on citrated blood sample<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Hypercoagulability versus platelet dysfunction | Predominance of hypercoagulability versus platelet dysfunction in beta-thalassemia patients | one year | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Regular screening of thalassemia patients | Evaluation of the significance of implementing regular screening of thalassemia patients for any possible hemostatic changes. | one year |