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

Study Overview
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Brief Summary
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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.

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

Conditions
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Thalassemic Syndromes

Intervention / Treatment
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* Other: Free α-Hb pool analysis


Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum 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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Design Details

Primary Purpose: Diagnostic
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Other: Blood sample<br>A blood collection is carry out in the three populations of patients during the day of their enrolment. | Other: Free α-Hb pool analysis<br> <br> |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Correlation between the pool of free α-Hb and genotype α and β -globin of the patient and if necessary with the genotype AHSP gene |  | Day 1 | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Correlation between the pool of free α-Hb and Hb value |  | Day 1 | 
| Correlation between the pool of free α-Hb and Mean Corpuscular Hb |  | Day 1 | 
| Correlation between the pool of free α-Hb and Mean Cell Volume |  | Day 1 | 
| Correlation between the pool of free α-Hb and percent of reticulocytes |  | Day 1 | 
| Correlation between the pool of free α-Hb and fetal Hb |  | Day 1 | 
| Correlation between the pool of free α-Hb and erythrocyte enzymes |  | Day 1 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Alpha-Hemoglobin Stabilizing Protein, alpha-Hb pool, thalassemias, diagnostic method, biomarker