Lipid Balance in Adult Sickle Cell Patients

Study of Lipid Balance in Adult Sickle Cell SS or SC Patients at Steady State and According to Clinical Phenotypes and During Acute Complications Acronym : "HDL2"

This study aims to describe and/or searches for, in cohorts of adult sickle cell anemia (SCA) and SC sickle cell patients living in the French West Indies and followed by SCD Reference and Competence Centers: 1-lipids profiles and associations at steady state with occurrence of sickle cell disease (SCD) complications, 2-lipids profile evolution during and after prospective acute complications (vasoocclusive crises (VOC) and priapism), 3-lipids profile variation (inter /intra individuals) during 4 prospective years, 4- Genetic primary modulators of SCD complications, 5- insulin resistance (HOMA), free fatty acids and glycerol dosages, 6- lipids enzymes, lipidome and functionality of HDL in sub-groups of SCD population.

• Cohorts of sickle cell disease patients including sickle cell anemia (SCA) and SC sickle cell patients living in Guadeloupe and Martinique and followed by the Sickle cell disease (SCD) Reference and Competence Centers of French West Indies.
• Lipid profile includes total cholesterol, HDL-cholesterol, non-HDL-cholesterol, LDL-cholesterol and triglycerides, apolipoprotein A-I and B.

Medical histories and prospective collection of SCD complications include retinopathy, deafness, tinnitus, osteonecrosis, leg ulcers, strokes, acute chest syndrome, VOC, priapism, pulmonary arterial hypertension (PAH) and PAH sd (echocardiography diagnosed when tricuspid regurgitant jet velocity ≥2.5 m/sec), kidney disease: chronic renal insufficiency and/or nephropathy.

• Objective 4: to describe genetic primary modulators of SCD complications: fetal hemoglobin, alpha-thalassemia, haplotypes of beta S gene.
• Objective 5 will be performed in the entire cohort at inclusion and during prospective complications (VOC, priapism).
• Objective 6 will be performed in a sub-group of 90 individuals (n=15 with VOC and n= 15 without VOC, n=15 with priapism and n=15 without priapism, n= 15 with pulmonary arterial hypertension syndrome (PAH Sd) and n=15 without PAH Sd), as well as in a subgroup of n = 15 patients prospectively experiencing VOC and n = 15 patients prospectively experiencing priapism.

A collection of plasma is performed to fulfill objective 6, as well as a collection of blood cells for later researches.

Inclusion Criteria:

• Aged from 18 years and over
• Be affected with Sickle cell anemia or SC sickle cell
• Living in French Caribbean Islands of Guadeloupe or Martinique and followed by physicians issued from a French West Indies Sickle Cell Reference or Competence Center
• At steady state in the last month (without acute complication)
• To have given a written consent after information on the study.

Exclusion Criteria:

• Other hemoglobinopathies than sickle cell disease
• Pregnancy or lactation
• Patient under judicial protection or without freedom
• Patient not affiliated with a social security system
• Patient hospitalized for transfusion or bleeding in the last 3 months