Bandeau Equipe 2

Transfusion and Pathologies of the Red Blood Cell

Contacts

France Pirenne Head France Pirenne

Tél.: +33-(0)1 56 72 76 00
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Our team, created in January 2015, is composed of 3 former entities: Etablissement Français du Sang (EFS), Inserm and UPEC. Our 3 entities are involved in red blood cell (RBC) disease pathophysiology and treatment, with a specific focus on sickle cell disease (SCD) and thalassemia. All 3 entities already worked with the referral center of genetic disease of RBC in the H. Mondor Hospital

Fig3 Page Equipe Noizat-Pirenne

 

The former entities are:

– The EFS team directed by Pr F. Pirenne (team leader of the Eq2), which has developed, since 2008 in the H.Mondor Hospital, research projects on transfusion risks in SCD patients and antibody mediated hemolysis in autoimmune haemolytic anemia.

– The Inserm team (ex Inserm U779) specialized in protein polymerisation, blood substitutes and rare RBC diseases. The projects of this team have historically been centred on the RBC, with much of the experimental work at the protein level. The themes include hemoglobin (Hb)-based blood substitutes, the Alpha-Hemoglobin Stabilizing Protein (AHSP), chaperone of alpha chains, and neuroglobin (Ngb).

– The UPEC team specialized in RBC genetic and physiopathology SCD.

 

 

 

The new projects of our group combine the expertise of all members with a specific focus on :

– mechanism of allo immunization against red blood cells

– pathophysiology of post-transfusion hemolysis in sickle cell disease

– role of dense red blood cells in the pathophysiology of sickle cell disease

– study of the free pool alpha in thalassemia and other red blood cell disease

Epidemiological, transversal and fundamental studies are developed on thesis themes.

Fig1 Page Equipe Noizat-Pirenne Fig2 Page Equipe Noizat-Pirenne

Our team is a member of the Laboratoire d’Excellence du Globule Rouge, GR-Ex (http://www.labex-grex.com)


Selected publications

Rakotoson MG, Di Liberto G, Audureau E, Habibi A, Fauroux C, Khorgami S, Hulin A, Loric S, Noizat-Pirenne F, Galacteros F, Bartolucci P. Biological parameters predictive of percent dense red blood cell decrease under hydroxyurea. Orphanet J Rare Dis. 2015 May 9;10(1):57. doi: 10.1186/s13023-015-0272-3.

pubmed/25956133

Noizat-Pirenne F, Habibi A, Mekontso-Dessap A, Razazi K, Chadebech P, Mahevas M, Vingert B, Bierling P, Galactéros F, Bartolucci P, Michel M. The use of rituximab to prevent severe delayed haemolytic transfusion reaction in immunized patients with sickle cell disease. Vox Sang. 2015 Apr;108(3):262-7.

pubmed/25523469

Vingert B, Tamagne M, Habibi A, Pakdaman S, Ripa J, Elayeb R, Galacteros F, Bierling P, Ansart-Pirenne H, Bartolucci P, Noizat-Pirenne F. Phenotypic differences of CD4+ T cells in response to red blood cell immunization in transfused sickle cell disease patients.Eur J Immunol. 2015 Mar 12. doi: 10.1002/eji.201445187.

pubmed/25763868

Burin des Roziers N, Chadebech P, Bodivit G, Guinchard E, Bruneel A, Dupré T, Chevret L, Jugie M, Gallon P, Bierling P, Noizat-Pirenne F. Red blood cell Thomsen-Friedenreich antigen expression and galectin-3 plasma concentrations in Streptococcus pneumoniae-associated hemolytic uremic syndrome and hemolytic anemia. Transfusion. 2014 Dec 30. doi: 10.1111/trf.12981

pubmed/25556575

Domingues-Hamdi E, Vasseur C, Fournier JB, Marden MC, Wajcman H, Baudin-Creuza V. Role of α-globin H helix in the building of tetrameric human hemoglobin: interaction with α-hemoglobin stabilizing protein (AHSP) and heme molecule. PLoS One. 2014 Nov 4;9(11):e111395.

pubmed/25369055

Arlet JB, Ribeil JA, Guillem F, Negre O, Hazoume A, Marcion G, Beuzard Y, Dussiot M, Moura IC, Demarest S, de Beauchêne IC, Belaid-Choucair Z, Sevin M, Maciel TT, Auclair C, Leboulch P, Chretien S, Tchertanov L, Baudin-Creuza V, Seigneuric R, Fontenay M, Garrido C, Hermine O, Courtois G. HSP70 sequestration by free α-globin promotes ineffective erythropoiesis in β-thalassaemia. Nature. 2014 Oct 9;514(7521):242-6.

pubmed/25156257

Silvy M, Tournamille C, Babinet J, Pakdaman S, Cohen S, Chiaroni J, Galactéros F, Bierling P, Bailly P, Noizat-Pirenne F. Red blood cell immunization in sickle cell disease: evidence of a large responder group and a low rate of anti-Rh linked to partial Rh phenotype. Haematologica. 2014 Jul;99(7):e115-7.

pubmed/24727821

Mellerio C, Farhat WH, Calvet D, Oppenheim C, Lefaucheur JP, Bartolucci P. Cerebral reorganization of language and motor control secondary to chronic hemispheric vasculopathy in a patient with homozygous sickle-cell disease. Am J Hematol. 2014 Jun;89(6):662-3.

pubmed/24668836

Vingert B, Tamagne M, Desmarets M, Pakdaman S, Elayeb R, Habibi A, Bernaudin F, Galacteros F, Bierling P, Noizat-Pirenne F, Cohen JL. Partial dysfunction of Treg activation in sickle cell disease. Am J Hematol. 2014 Mar;89(3):261-6.

pubmed/24779034

Gueguen A, Mahevas M, Nzouakou R, Hosseini H, Habibi A, Bachir D, Brugière P, Lionnet F, Ribei JA, Godeau B, Girot R, Ibrahima V, Calvet D, Galactéros F, Bartolucci P. Sickle-cell disease stroke throughout life: a retrospective study in an adult referral center. Am J Hematol. 2014 Mar;89(3):267-72.

pubmed/24779035

Kiger L, Vasseur C, Domingues-Hamdi E, Truan G, Marden MC, Baudin-Creuza V. Dynamics of α-Hb chain binding to its chaperone AHSP depends on heme coordination and redox state. Biochim Biophys Acta. 2014 Jan;1840(1):277-87.

pubmed/24060751

Russo R, Giordano D, di Prisco G, Hui Bon Hoa G, Marden MC, Verde C, Kiger L. Ligand-rebinding kinetics of 2/2 hemoglobin from the Antarctic bacterium Pseudoalteromonas haloplanktis TAC125. Biochim Biophys Acta. 2013 Sep;1834(9):1932-8.

pubmed/23429181

Ghedira ES, Pissard S. Low fetal hemoglobin rates in patients carrying Thai (δβ)0-deletion and Turkish (δβ)0-deletion/inversion strengthen the hypothesis that the 5'δ BCL11A binding site plays a major role in its fetal hemoglobin inhibitory regulation. Response to "The 12.6 kb-deletion in the β-globin gene cluster is the known Thai/Vietnamese (δβ)0-thalassemia commonly found in Southeast Asia". Haematologica. 2013 Sep;98(9):e119-20.

pubmed/24006413

Bartolucci P, Brugnara C, Teixeira-Pinto A, Pissard S, Moradkhani K, Jouault H, Galacteros F. Erythrocyte density in sickle cell syndromes is associated with specific clinical manifestations and hemolysis. Blood. 2012 Oct 11;120(15):3136-41.

pubmed/22919030

Yazdanbakhsh K, Ware RE, Noizat-Pirenne F. Red blood cell alloimmunization in sickle cell disease: pathophysiology, risk factors, and transfusion management. Blood. 2012 Jul 19;120(3):528-37.

pubmed/22563085

Brillet T, Marden MC, Yeh JI, Shen TJ, Ho NT, Kettering R, Du S, Vasseur C, Domingues-Hamdi E, Ho C, Baudin-Creuza V. Interaction of haptoglobin with hemoglobin octamers based on the mutation αAsn78Cys or βGly83Cys.Am J Mol Biol. 2012 Apr 1;2(1):1-10.

pubmed/23847747