Éditeur(s) : HAL CCSD Wiley
Résumé : International audience
Sickle cell anemia (SS) is characterized by a reduced cerebral microvascular oxygen saturation (cerebral TOI), which is not associated with hemoglobin concentration. Cerebral TOI has never been studied in sickle cell-hemoglobin C disease (SC). We focused on the relationships between hemorheological alterations and cerebral TOI in sickle cell patients with no cerebral vasculopathy and on the usefulness of TOI variability to assess the cerebral vasomotion activity. The blood rheological profile, the level of cerebral TOI (spatial resolved spectroscopy) and the cerebral TOI variability, which reflects vasomotion activity, were compared between 20 healthy subjects (AA), 21 SC patients, and 21 SS patients. Cerebral TOI exhibited the following order: AA > SC > SS. The low cerebral TOI in SS patients was related to red blood cell aggregation and deformability properties. The cerebral TOI variability of SS and SC patients was increased above healthy values and vasomotion activity was negatively associated with the reduced cerebral TOI in SS patients. We demonstrated that (1) blood rheology could be involved in the reduced cerebral TOI in SS patients but not in SC patients; (2) vasomotion activity is increased in SS and SC patients to compensate for the reduced cerebral TOI.
ISSN: 0361-8609

inserm-00838966
http://www.hal.inserm.fr/inserm-00838966
http://www.hal.inserm.fr/inserm-00838966/document
http://www.hal.inserm.fr/inserm-00838966/file/2013_Waltz_American_Journalsur Hematology.pdf
DOI : 10.1002/ajh.23318

Éditeur(s) : HAL CCSD Public Library of Science
Résumé : International audience
Sickle cell anemia (SCA) and hemoglobin SC (HbSC) disease are the two most common forms of sickle cell disease (SCD), a frequent hemoglobinopathy which exhibits a highly variable clinical course. Although high levels of microparticles (MPs) have been consistently reported in SCA and evidence of their harmful impact on the SCA complication occurrences have been provided, no data on MP pattern in HbSC patients has been reported so far. In this study, we determined and compared the MP patterns of 84 HbSC and 96 SCA children, all at steady-state, using flow cytometry. Most of circulating MPs were derived from platelets (PLTs) and red blood cells (RBCs) in the two SCD syndromes. Moreover, we showed that HbSC patients exhibited lower blood concentration of total MPs compared to SCA patients, resulting mainly from a decrease of MP levels originated from RBCs and to a lesser extent from PLTs. We did not detect any association between blood MP concentrations and the occurrence of painful vaso-occlusive crises, acute chest syndrome and pulmonary hyper-tension in both patient groups. We also demonstrated for the first time, that whatever the considered genotype, RBC-derived MPs exhibited higher externalized phosphatidylserine level and were larger than PLT-derived MPs.
ISSN: 1932-6203

hal-01668322
https://hal.univ-antilles.fr/hal-01668322
https://hal.univ-antilles.fr/hal-01668322/document
https://hal.univ-antilles.fr/hal-01668322/file/journal.pone.0177397.pdf
DOI : 10.1371/journal.pone.0177397

Éditeur(s) : HAL CCSD ACSM
Résumé : International audience
A 26-yr-old man with sickle cell trait (SCT) suddenly lost visual acuity in the left eye after a cycling race in hot tropical environment. The cause was massive central retinal vein occlusion (CRVO) with hemorrhaging that rapidly worsened to neovascular glaucoma. Although medically treated, the eye is now marked by total retinal detachment. Cardiovascular function assessment shows no electrocardiographic abnormalities, no anomaly in the supra-aortic tree, and no evidence of structural heart disease. Although normal coagulation markers values (i.e., activated partial thromboplastin time, prothrombin time, fibrinogen concentration, antithrombin III, factor V, proteins C and S) were observed 2.5 months after the clinical event, a transesophageal echocardiogram performed few hours after the incident revealed the presence of four thrombi in the left atrium suggesting a postexercise hypercoagulable state at that time. Hemorheological measurements at distance of the events demonstrated high red blood cell rigidity at baseline. Therefore, marked blood rheological impairment and activation of the coagulation pathway in response to the heavy and prolonged cycling race could have promoted CRVO in this cyclist carrying SCT. These data suggest that SCT could be considered as a risk factor for significant ocular complications when severe exercise is performed and support the idea that SCT is a contributing factor in blood rheology and vascular dysfunctions.
Medicine & Science in Sports & exercise

Droits : info:eu-repo/semantics/OpenAccess
hal-00698572
https://hal.univ-antilles.fr/hal-00698572
https://hal.univ-antilles.fr/hal-00698572/document
https://hal.univ-antilles.fr/hal-00698572/file/Hedreville_et_al_revised_MSSE-D-08-00064R2.pdf
DOI : 10.1249/MSS.0b013e31818313d0

Éditeur(s) : HAL CCSD
Résumé : International audience
This study compared the hemorheological profile at rest and in response to a short supramaximal exercise test between sickle cell trait (SCT) carriers and a control group. Eight SCT carriers and eight control subjects performed a ramp exercise test on a cycle ergometer conducted to maximal oxygen uptake ðV_ O2maxÞ: One week later, they performed a supramaximal exercise test consisting of pedaling for 1 min at 110% V_ O2max: Blood viscosity (gb), plasma viscosity (gp), hematocrit (Hct) and red blood cell (RBC) rigidity were assessed at rest, at the end of exercise and at the 15th, 30th and 60th min of recovery. Exercise increased gb, gp and Hct above resting values in both groups and these parameters remained higher until the 15th or 30th min of recovery as compared to resting values. RBC rigidity was unchanged from baseline values in both groups during exercise and recovery. No difference was observed between the two groups for gp and Hct but gb and RBC rigidity were higher in the SCT carriers at every time point compared with the control group. The higher RBC rigidity and gb found in SCT carriers at rest and in response to a brief supramaximal exercise might constitute a risk factor for microcirculatory complications. Indeed, a short supramaximal exercise test may not be completely inoffensive for SCT carriers.
EUROPEAN JOURNAL OF APPLIED PHYSIOLOGY

hal-00706158
https://hal.univ-antilles.fr/hal-00706158
DOI : 10.1007/s00421-006-0155-3

Éditeur(s) : HAL CCSD IOS Press
Résumé : International audience
This study tested the hypothesis that trained sickle cell trait (SCT) carriers are not subjected to greater risk of rhabdomyolysis or renal failure in response to moderate submaximal exercise than subjects with normal hemoglobin (CONT). Blood markers in 11 trained SCT carriers and 12 control counterparts were measured before and after 40 min of exercise at 55% of peak power output (Ppeak) conducted in thermoneutral environment. Body weights decreased with exercise in the same proportion in the two groups (from 65.1 ± 7.0 kg to 64.1 ± 7.0 kg and from 70.2 ± 6.6 to 68.6 ± 6.6 kg at the end of exercise in SCT and CONT, respectively). Heart rate and rectal temperature increased in the two groups in response to exercise, but the groups remained closely matched. Serum urea, CRP, CK and LDH were similar in the two groups and remained unchanged in response to exercise. Creatinine, Na + , K + , Cl − and myoglobin concentrations increased above baseline in response to exercise, with changes of the same magnitude in the two groups. In summary, the results of the present study suggest that moderate submaximal exercise is not unsafe from a biochemical point of view for sportsmen carrying SCT.
ISSN: 1386-0291

hal-01163943
https://hal.univ-antilles.fr/hal-01163943
DOI : 10.3233/CH-2011-1524

Résumé : La drépanocytose est une hémoglobinopathie aux formes et aux sévérités cliniques très hétérogènes qui affectent de nombreux organes chez les patients touchés. Pour cette raison le terme de syndrome drépanocytaire majeur est généralement employé. Tous les syndromes drépanocytaires majeurs ont en commun une mutation ponctuelle du gène β-globine appelée mutation βs . Cette mutation βs conduit à la synthèse de la protéine d'hémoglobine S (HbS) caractérisée par sa capacité à polymériser dans sa forme désoxygénée. La polymérisation de l'HbS est l'élément déclencheur de la falciformation du globule rouge et conduit à de nombreuses altérations hématologiques et hémorhéologiques. Ces dernières sont à l'origine de troubles de l'hémodynamique, de l'oxygénation sanguine et de la perfusion tissulaire en oxygène. Il en résulte de nombreuses complications aigues (crise vaso-occlusive, accident vasculaire cérébral, syndrome thoracique aigu etc.) et/ou chroniques (glomérulopathie, ostéonécrose de la tête fémorale etc.) causés par un défaut d'oxygénation des tissus.
Sickle cell anemia is a hemoglobinopathy forms and severities very heterogeneous clinical affecting many organs in affected patients. For this reason the term major sickle cell syndrome is generally used. All major sickle cell syndromes have a common point mutation in the gene called β-globin mutation βs. This βs mutation leads to the synthesis of the protein hemoglobin S (HbS) is characterized by its ability to polymerize in its deoxygenated form. The polymerization of HbS is the trigger for the sickling of red blood cells and leads to many Hematological and hemorheological. These are at the origin of disorders hemodynamics, blood oxygenation and tissue perfusion with oxygen. This results in many acute complications (vaso-occlusive crisis, stroke, acute chest syndrome, etc..) And / or chronic (glomerulopathy, osteonecrosis of the femoral head etc.). Caused by a defect of tissue oxygenation.

http://www.theses.fr/2012AGUY0563/document