The Change of Coagulation Markers in Children With β-thalassemia Disease After Stem Cell Transplantation

Study Overview
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Brief Summary
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Hypercoagulable state is well recognized in patients with β-thalassemia. Evidences of hypercoagulability include abnormal expression of phosphatidylserine on red blood cell (rbc) surface and consequent increased platelet activation and thrombin generation. In addition, a reduction of anticoagulants i.e. proteins C and S and antithrombin (AT) was demonstrated. However, coagulable state in patients with β-thalassemia following stem cell transplantation (SCT) has not been characterized.

Detailed Description
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Hypercoagulable state is well recognized in patients with β-thalassemia. Evidences of hypercoagulability include abnormal expression of phosphatidylserine on red blood cell (rbc) surface and consequent increased platelet activation and thrombin generation. In addition, a reduction of anticoagulants i.e. proteins C and S and antithrombin (AT) was demonstrated. However, coagulable state in patients with β-thalassemia following stem cell transplantation (SCT) has not been characterized.Therefore, the objective is to compare coagulation markers and anticoagulants among β-thalassemics with and without SCT and normal control (NC).The subjects will be classified into 3 groups; β-thalassemia post SCT (Thal-SCT), β-thalassemia treated with regular transfusion (Thal-RT) and NC. Blood samples will be tested for annexin V (an index of abnormal expression of phosphatidylserine on rbc surface), markers of activation of coagulation system (thrombin antithrombin complex (TAT), prothrombin fragment (F1+2), and D-dimer) and anticoagulants (proteins C and S and AT).

Official Title
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The Change of Coagulation Markers in Children With β-thalassemia Disease After Stem Cell Transplantation

Conditions
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Thalassemia

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Group 1: beta thalassemia major or beta thalassemia / Hb E who receive regular transfusion therapy (Thal- RT). The baseline Hct was more than 24% for at least 6 months. Group 2: beta thalassemia major or beta thalassemia / Hb E post SCT (Thal-SCT) who were discontinued immunosuppressive drugs. Group 3: Normal children (NC) who had normal Hb/Hct and MCV for age Exclusion Criteria: Children with beta thalassemia major or beta thalassemia / Hb E who have co-diseases such as immune hemolytic anemia, infection, or inflammatory diseases

Ages Eligible for Study
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Minimum Age: 1 Year
Maximum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| 1<br>Normal control | |
| 2<br>B thalassemia regular transfusion | |
| 3<br>B thalassemia post transplantation | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Level of protein C,S and AT, TAT, P1+2 and D-dimer |  | 3 years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Coagulation markers, thalassemia disease post SCT