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| ID | Type | Description | Link |
|---|---|---|---|
| 2009-18 |
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The results will allow us to evaluate the role of MP in the thrombo-embolic risk observed in thalassemic patients and to underline a possible difference between TM and TI. The in vitro and in vivo study of MP in erythrocytes concentrates is a new approach to explore the consequence of transfusion in polytransfused patients. Finally, the identification of a possible relationship between the oxidative stress and the production of MP may lead to the development of specific therapeutical approaches
Microparticles (MP) are intact vesicles derived from cell membranes which arise mainly through cell membrane activation processes and from apoptosis. MP originating from platelets, endothelial cells and monocytes have been most extensively studied, though similar particles can arise from red cells and granulocytes. The ability to form microparticles is an essential part of physiological coagulation.However, MP may play an important procoagulant role in several diseases including sickle cell disease, and paroxysmal nocturnal haemoglobinuria (PNH).
Several studies reported the presence of MP in TI and their potential role in the hypercoagulable state. The investigators propose in this study to investigate the presence and origin of MP in TM patients.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| TM patients | Active Comparator | thalassemia major (TM) Need transfusion for survive |
|
| TI patients | Active Comparator | thalassemia intermedia (TI) Patients with TI have a milder clinical phenotype than those with TM |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Physiopathology | Other | Three sequential biological evaluations will be performed for each patient and will consist in :
In vitro production of MP of transfused red blood cells origin will also be evaluated in erythrocytes concentrates during the storage of the units. |
| Measure | Description | Time Frame |
|---|---|---|
| Relationship between TM and TI |
| 36 months |
| Measure | Description | Time Frame |
|---|---|---|
| Investigate the mechanisms of the elevated production of MP in thalassemias | Studying the correlation between the number, the activity of erythrocytes and platelets derived-MP and the hemolysis, the dyserythropoiesis, the oxidative stress and iron overload markers. | 36 months |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Isabelle Thuret, Doctor | APHM | Study Director |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| APHM | Marseille | 13 | France |
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| ID | Term |
|---|---|
| D017086 | beta-Thalassemia |
| ID | Term |
|---|---|
| D013789 | Thalassemia |
| D000745 | Anemia, Hemolytic, Congenital |
| D000743 | Anemia, Hemolytic |
| D000740 | Anemia |
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|
| Physiopathology | Other | Three sequential biological evaluations will be performed for each patient and will consist in :
|
|
| D006402 |
| Hematologic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
| D006453 | Hemoglobinopathies |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |