Evaluation of the Free α-hemoglobin Pool in the Red Blood Cells : Prognostic Marker and Severity Index in Thalassemic Syndromes

Evaluation of the Free α-hemoglobin Pool in the Red Blood Cells : Prognostic Marker and Severity Index in Thalassemic Syndromes

Our project aims to assess and validate an innovative prognostic and diagnostic test in order to i) define a severity index of the β-thalassemia according to the free α-Hb pool for a better management of the patient; ii) differentiate the α-thalassemia and β-thalassemia and iii) follow the evolution of this pool in response to treatment of patients. In the future, this prognostic test based on the measurement of free α-Hb pool may be suitable for routine laboratory practice. To carry out this project, cohort of 85 thalassemic patients and a group of 50 healthy volunteers have been recruited.

Inclusion Criteria Patient

• Age ≥ 18 years
• Written informed consent
• Affiliation to social security
• Known Hb Phenotypes
• Biological phenotype corresponding to a classical picture of thalassemia

Healthy volunteers

• Age ≥ 18 years
• Written informed consent
• Affiliation to social security

Exclusion Criteria Patient

• Transfusion for less than 3 months
• Chronic active viral disease: hepatitis B, C, HIV
• Ongoing infections or known inflammatory diseases
• Hyper or Hypothyroidism known or subject treated by Levothyrox
• Active pathology or tumor remission for less than 5 years
• Oral corticosteroid
• Hemoglobinopathy other than thalassemia for patients
• Treatment by Hydroxyurea for more than 3 months
• Treatment by stimulating agent erythropoiesis for longer than 3 months

Healthy volunteers

• Transfusion for less than 3 months
• Chronic active viral disease: hepatitis B, C, HIV
• Ongoing infections or known inflammatory diseases
• Hyper or Hypothyroidism known or subject treated by Levothyrox
• Active pathology or tumor remission for less than 5 years
• Oral corticosteroid
• Genetically related to person with hemoglobinopathy