Sickle cell disease

French national reference center for major sickle cell syndromes and other rare pathologies of the red blood cell and erythropoiesis

drapeau françaisLogo Drepano HEGPOur internal medicine department at the Georges-Pompidou European hospital was certified as an expert center in 2008 in order to improve the management of adult patients with sickle cell disease in western Paris as well as other rare diseases of the red blood cell and erythropoiesis : G6PD deficiency, hereditary spherocytosis, thalassemia, etc.

It allows, among other things, to ensure the transition of the pediatric cohort of the Necker hospital of approximately 600 patients.

Professor Jean-Benoît Arlet has been responsible for the reference center since 2008.

Logo sickle cell center HEGPThe center develops fundamental research projects (at the Imagine laboratory, Necker), but also epidemiological and therapeutic trials on new treatments.

A therapeutic education program is very active to support patients and their families. Expert patients and patient associations are integrated into our team with the aim of constantly improving care.

Bandeau vidéo drépano journée mondiale HEGP

2022 world sickle cell day

Vignette Video journée mondiale drepanocytose

2021 world sickle cell day

Medical team

Photo Arlet HEGP DREPANO

Pr Jean-Benoît Arlet
MD, PhD

Contact us

Phone. +33 1 56 09 53 41

> Send an email

In case of emergency

The missions of the center are :

  • Caring for sickle cell patients in crisis in the emergency room and in hospitalization.
  • Constantly improve the quality of patient care and their quality of life : reception, speed of care, particularly in emergencies, comprehensive care, listening …
  • Prevent and treat chronic complications of red blood cell diseases (including sickle cell disease) in consultation and day hospitalization.
  • To be a referral center for complex diagnoses of red blood cell diseases (membrane diseases, enzymatic, etc.), complex anemias, and their management.
  • Find innovative ways to improve patient care: therapeutic education (TPE) ; improvement of the hospital setting ; technical solution to improve the perfusion of sickle cell patients, transfusions, compliance with disease-modifying treatments; patient involvement in our meetings, therapeutic and research protocols; involvement of new actors in care (volunteers, patient visitors and hospital schools for example, associations), website, social networks…
  • Training of doctors and paramedics in red blood cell diseases
  • Develop therapeutic education
  • Improving the child-adult transition
  • Raise awareness of these diseases among the general public and health actors
  • Organize the medico-social care of these diseases
  • Research mission: Advance knowledge of the physiopathology of hemoglobinopathies with the aim of rapid therapeutic benefits for patients.
  • Clinical, epidemiological and basic research.
Photo Arlet HEGP DREPANO

Pr Jean-Benoît Arlet
MD, PhD

Photo Pr Brigitte Ranque Drepano HEGP

Pr Brigitte Ranque
MD, PhD

photo identité homme

Dr Geoffrey Cheminet
MD

Photo Dr Edouard Flamarion Drepano HEGP

Dr Edouard Flamarion
MD

photo identité homme

Dr Emmanuel Lafont
MD

Photo Dr Adrien Michon Drepano HEGP

Dr Adrien Michon
MD

Photo Anne Corbasson

Anne Corbasson
Coordinating nurse, TPE, research

Carole Barthélémy
TPE referring nurse

Marie Charmettan
TPE referring nurse

photo identité homme

Djiry Niakate
TPE referring caregiver

Photo Tong Drepano

Evelyne Tong
TPE referring caregiver

Marie Bernard
Psychologist

Mélissa Alègre
Dietitian

Amélie Mière Perrin
Social worker

Photo Khimoud Drepano

Djamal Khimoud
Clinical research assistant

Photo Namaoui Drepano

Walid Namaoui
Clinical research assistant

Photo Akpamoli Drepano

Eniade Akpamoli
Secretary

Photo Bakhtaoui Drepano

Anissa Bakhtaoui
Secretary

Hadji Dem
Secretary

Photo Isabelle Leon drepano

Isabelle Léon
Secretary

Cinnamone Manjakavelo
Secretary

Logo ETP Drepano HEGP EvadPublic : People with sickle cell disease from the age of 16, followed in the service, as well as their carers.

Contact at HEGP : Anne Corbasson

> Send an email

RESEARCH

  • Oxyckle (2017 national PHRC) : Oxygen therapy for the management of vaso-occlusive crises in adult patients with sickle-cell disease : a multicenter, double blind, placebo controlled randomized trial – Ongoing
  • DREPARIC (2017 national PHRC) : Attenuated conditioning genoidentical transplantation in adult sickle cell disease – Ongoing
  • MOOD (2018 national PHRC) : Analgesia with MethOxyflurane in the Initial Management of VasoOCclusive Sickle Cell Crises in the Emergency Room – Ongoing
  • DREPADO (coordination Lyon, PREPS 2017) : Impact of a pediatric-adult transition program on the health status of adolescents with sickle cell disease: a randomized controlled trial – Ongoing
  • Agios laboratory : Phase 2/3 multicenter, double-blind, randomized, placebo-controlled study to evaluate the efficacy of Mitapivat in patients with sickle cell disease – Start of inclusion summer 2022
  • STAND (Novartis laboratory) : Study of two doses of crizanlizumab versus placebo in adolescent and adult sickle cell disease patients – Ongoing, completed inclusions
  • PERFID : Assisting peripheral PERFusion by infrared venous illumination in the sickle cell patient in crisis: A randomized open-label controlled study – Ongoing, inclusions completed by March 2022
  • SIKAMIC (Addmedica laboratory) : SIklos on Kidney function and AlbuMInuria Clinical trial) : étude de phase 3 internationale, Multicentre randomized double-blind placebo-controlled study to evaluate the effect on albuminuria of 6 months treatment with hydroxycarbamide (Siklos®) or a placebo in adults with sickle cell disease – Ongoing, still open in 2022

EDUCATION

2022

– [Gallstone complications in sickle cell patients].
Rambaud E, Ranque B, Pouchot J, Arlet JB.
Rev Med Interne, 2022 Jun 7, PMID: 35688668 DOI: 10.1016/j.revmed.2022.05.006

– Blood exchange transfusion with dexamethasone and Tocilizumab for management of hospitalized patients with sickle cell disease and severe COVID-19: Preliminary evaluation of a novel algorithm.
De Luna G, Habibi A, Odièvre MH, Guillet H, Guiraud V, Cougoul P, Carpentier B, Loko G, Guichard I, Ourghanlian C, Pawlotsky JM, Mahevas M, Limal N, Michel M, Mekontso-Dessap A, Arlet JB, Bartolucci P.
Am J Hematol, 2022 Jul, PMID: 35385170 PMCID: PMC9073971 DOI: 10.1002/ajh.26563

Risk factors for severe COVID-19 in hospitalized sickle cell disease patients: A study of 319 patients in France.
Arlet JB, Lionnet F, Khimoud D, et al.
Am J Hematol, 2022 Mar 1, PMID: 34882837 PMCID: PMC9011445 DOI: 10.1002/ajh.26432

– Estimating the risk of child mortality attributable to sickle cell anaemia in sub-Saharan Africa: a retrospective, multicentre, case-control study.
Ranque B, Kitenge R, Ndiaye DD, et al.
Lancet Haematol, 2022 Mar, PMID: 35240076 DOI: 10.1016/S2352-3026(22)00004-7

2021

– Effect of hydroxyurea exposure before puberty on sperm parameters in males with sickle cell disease.
Joseph L, Jean C, Manceau S, Chalas C, Arnaud C, Kamdem A, Pondarré C, Habibi A, Bernaudin F, Allali S, de Montalembert M, Boutonnat-Faucher B, Arlet JB, Koehl B, Cavazzana M, Ribeil JA, Lionnet F, Berthaut I, Brousse V.
Blood, 2021 Feb 11, PMID: 32976551 DOI: 10.1182/blood.2020006270

Deficient mitophagy pathways in sickle cell disease.
Martino S, Arlet JB, Odièvre MH, Jullien V, Moras M, Hattab C, Lefebvre T, Gouya L, Ostuni MA, Lefevre SD, Le Van Kim C.
Br J Haematol, 2021 Jun, PMID: 33754349 DOI: 10.1111/bjh.17416

– Epidemiology and disease burden of sickle cell disease in France: A descriptive study based on a French nationwide claim database.
Leleu H, Arlet JB, Habibi A, Etienne-Julan M, Khellaf M, Adjibi Y, Pirenne F, Pitel M, Granghaud A, Sinniah C, De Montalembert M, Galacteros F.
PLoS One, 2021 Jul 9, PMID: 34242255 PMCID: PMC8270152 DOI: 10.1371/journal.pone.0253986

– Syndrome thoracique aigu chez le patient drépanocytaire 
Cheminet G, A Mékontso-Dessap, B Ranque, J Pouchot, JB Arlet .
Rev Med Interne, 2021

Impact of sickle cell disease on patients » daily lives, symptoms reported, and disease management strategies: Results from the international Sickle Cell World Assessment Survey (SWAY).
Ifeyinwa Osunkwo,Biree Andemariam,Caterina P. Minniti,Baba P. D. Inusa,Fuad El Rassi,Beverley Francis-Gibson,Alecia Nero,Cassandra Trimnell,Miguel R. Abboud,Jean-Benoît Arlet,Raffaella Colombatti,Mariane de Montalembert,Suman Jain,Wasil Jastaniah,Erfan Nur,Marimilia Pita,Laurie DeBonnett,Nicholas Ramscar,Tom Bailey,Olivera Rajkovic-Hooley,John James
Am J Hematol, 2021 Apr 1, PMID: 33264445 PMCID: PMC8248107 DOI: 10.1002/ajh.26063

– Cell-derived microparticles and sickle cell disease chronic vasculopathy in sub-Saharan Africa: A multinational study.
Dembélé AK, Lapoumeroulie C, Diaw M, Tessougue O, Offredo L, Diallo DA, Diop S, Elion J, Colin-Aronovicz Y, Tharaux PL, Jouven X, Romana M, Ranque B, Le Van Kim C.
Br J Haematol, 2021 Feb, PMID: 33249569 DOI: 10.1111/bjh.17242

– Psychosocial risk factors for increased emergency hospital utilization by sickle cell disease patients: a systematic review protocol.
Jean-Simon Rech, Prunelle Getten, Nathalie Dzierzynski, François Lionnet, Pierre-Yves Boëlle, Brigitte Ranque, Olivier Steichen
JBI Evid Synth, 2021 Mar, PMID: 33725714 DOI: 10.11124/JBIES-20-00041

2020

XPO1 regulates erythroid differentiation and is a new target for the treatment of β-thalassemia.
Guillem, F ; Dussiot, M ; Colin, E ; Suriyun, T ; Arlet, JB ; Goudin, N & al
Haematologica, 2020 Jun 5, PMID: 33054049 PMCID: PMC7556489 DOI: 10.3324/haematol.2018.210054

Prognosis of patients with sickle cell disease and COVID-19: a French experience.
Jean-Benoît Arlet, Gonzalo de Luna, Djamal Khimoud, Marie-Hélène Odièvre, Mariane de Montalembert, Laure Joseph, Christelle Chantalat-Auger, Edouard Flamarion, Pablo Bartolucci, François Lionnet, Sebastien Monnier, Cécile Guillaumat, Aline Santin
Lancet Haematol, 2020 Sep, PMID: 32563282 PMCID: PMC7302791 DOI: 10.1016/S2352-3026(20)30204-0

XPO1 regulates erythroid differentiation and is a new target for the treatment of β-thalassemia.
Flavia Guillem, Michaël Dussiot, Elia Colin, Thunwarat Suriyun, Jean Benoit Arlet, Nicolas Goudin, Guillaume Marcion, Renaud Seigneuric, Sebastien Causse, Patrick Gonin, Marc Gastou, Marc Deloger, Julien Rossignol, Mathilde Lamarque, Zakia Belaid Choucair, Emilie Fleur Gautier, Sarah Ducamp, Julie Vandekerckhove, Ivan C Moura, Thiago Trovati Maciel, Carmen Garrido, Xiuli An, Patrick Mayeux , Narla Mohandas, Geneviève Courtois, Olivier Hermine
Haematologica, 2020 Sep 1, PMID: 33054049 PMCID: PMC7556489 DOI: 10.3324/haematol.2018.210054

Primary Hyperparathyroidism in Sickle Cell Disease: An Unknown Complication of the Disease in Adulthood.
Elsa Denoix, Charlène Bomahou, Lorraine Clavier, Jean-Antoine Ribeil, François Lionnet, Pablo Bartolucci, Marie Courbebaisse, Jacques Pouchot, Jean-Benoît Arlet
J Clin Med, 2020 Jan 22, PMID: 31979085 PMCID: PMC7073651 DOI: 10.3390/jcm9020308

Usefulness of azacitidine therapy in a sickle cell disease patient with myelodysplastic syndrome.
Gonzalo De Luna, Luc Darnige, Stéphane Roueff, Thierry Peyrard, Jacques Pouchot, Jean-Benoît Arlet
Ann Hematol, 2020 Mar, PMID: 32006149 DOI: 10.1007/s00277-020-03940-8

Impact of a paediatric-adult care transition programme on the health status of patients with sickle cell disease: study protocol for a randomised controlled trial (the DREPADO trial).
Delphine Hoegy, Nathalie Bleyzac, Alexandra Gauthier-Vasserot, Giovanna Cannas, Angélique Denis, Arnaud Hot, Yves Bertrand, Pauline Occelli, Sandrine Touzet, Claude Dussart, Audrey Janoly-Dumenil & DREPADO study group
Trials, 2020 Feb 10, PMID: 32039737 PMCID: PMC7008523 DOI: 10.1186/s13063-019-4009-9

[Advances in sickle cell disease treatments: Towards targeted therapies].
J-B Arlet
Rev Med Interne, 2020 Feb, PMID: 31771774 DOI: 10.1016/j.revmed.2019.11.001

[Sickle cell trait complications: A case series of 6 patients].
C Marcombes, E Lafont, V Jullien, E Flamarion, J Dion, N Costedoat-Chalumeau, J Pouchot, J B Arlet
Rev Med Interne, 2020 Sep, PMID: 32768266 DOI: 10.1016/j.revmed.2020.04.019

Automated RBC Exchange has a greater effect on whole blood viscosity than manual whole blood exchange in adult patients with sickle cell disease.
Nassim Ait Abdallah, Philippe Connes, Gaetana Di Liberto, Lucile Offredo, Jean Louis Beaumont, Dehbia Menouche, Karima Debbache, Amna Jebali, Anoosha Habibi, France Pirenne, Frédéric Galacteros, Brigitte Ranque, Pablo Bartolucci
Vox Sang, 2020 Nov, PMID: 32965032 DOI: 10.1111/vox.12990

2019

Comparison between Adult Patients with Sickle Cell Disease of Sub-Saharan African Origin Born in Metropolitan France and in Sub-Saharan Africa.
Honsel, V ; Khimoud, D ; Ranque, B ; Offredo, L ; Joseph, L ; Pouchot, J & al
J Clin Med, 2019 Dec 9, PMID: 31835320 PMCID: PMC6947353 DOI: 10.3390/jcm8122173

Trajectories of Biological Values and Vital Parameters: An Observational Cohort Study of Adult Patients with Sickle Cell Disease Hospitalized for a Non-Complicated Vaso-Occlusive Crisis.
Veil, R ; Bussy, S ; Looten, V ; Arlet, JB ; Pouchot, J ; Jannot, AS & al
J Clin Med, 2019 Sep 19, PMID: 31546961 PMCID: PMC6780589 DOI: 10.3390/jcm8091502

[Opioid-induced adrenal insufficiency: Case report and synthesis of the literature].
Flamarion, E ; Saada, N ; Khellaf, M ; Michon, A ; Passeron, A ; Pouchot, J & al
Rev Med Interne, 2019 Nov, PMID: 31444021 DOI: 10.1016/j.revmed.2019.07.003

A Phase 3 Randomized Trial of Voxelotor in Sickle Cell Disease.
Vichinsky, E ; Hoppe, CC ; Ataga, KI ; Ware, RE ; Nduba, V ; El-Beshlawy, A & al
N Engl J Med, 2019 Aug 8, PMID: 31199090 DOI: 10.1056/NEJMoa1903212

Sotatercept, a novel transforming growth factor β ligand trap, improves anemia in β-thalassemia: a phase II, open-label, dose-finding study.
Cappellini, MD ; Porter, J ; Origa, R ; Forni, GL ; Voskaridou, E ; Galactéros, F & al
Haematologica, 2019 Mar, PMID: 30337358 PMCID: PMC6395345 DOI: 10.3324/haematol.2018.198887

Plasma histamine elevation in a large cohort of sickle cell disease patients.
Allali, S ; Lionnet, F ; Mattioni, S ; Callebert, J ; Stankovic Stojanovic, K ; Bachmeyer, C & al
Br J Haematol, 2019 Jul, PMID: 30924132 DOI: 10.1111/bjh.15900

Comparison of methods for early-readmission prediction in a high-dimensional heterogeneous covariates and time-to-event outcome framework.
Bussy, S ; Veil, R ; Looten, V ; Burgun, A ; Gaïffas, S ; Guilloux, A & al
Br J Haematol, 2019 Jul, PMID: 30924132 DOI: 10.1111/bjh.15900

– Prevalence and correlates of growth failure in young African patients with sickle cell disease.
Alexandre-Heymann L, Dubert M, Diallo DA, Diop S, Tolo A, Belinga S, Sanogo I, Diagne I, Wamba G, Boidy K, Ly ID, Kamara I, Traore Y, Offredo L, Jouven X, Ranque B.
Br J Haematol, 2019 Jan, PMID: 30467843 DOI: 10.1111/bjh.15638

2018

High bone mineral density in sickle cell disease: Prevalence and characteristics.
De Luna G, Ranque B, Courbebaisse M, Ribeil JA, Khimoud D, Dupeux S, Silvera J, Offredo L, Pouchot J, Arlet JB.
Bone, 2018 May, PMID: 29428552 DOI: 10.1016/j.bone.2018.02.003

2017

– Roles of APOL1 G1 and G2 variants in sickle cell disease patients: kidney is the main target.
Kormann R, Jannot AS, Narjoz C, Ribeil JA, Manceau S, Delville M, Joste V, Prié D, Pouchot J, Thervet E, Courbebaisse M, Arlet JB.
Br J Haematol, 2017 Oct, PMID: 28699644 DOI: 10.1111/bjh.14842

  • Organization of the Francophone International Forum on Sickle Cell Disease in African Countries (FIFDA) every year since 2021 bringing together more than 500 doctors from Europe and Africa (www.drep-afrique.org).
  • National Sickle Cell Day at Tenon Hospital 20 min. Therapeutic news in sickle disease Pr JB ARLET 20 min 200 people
  • Paris hospitals medical society day :
    – Prevalence and pathophysiology of sickle cell disease. Pr JB ARLET 30 min 80 people
    – Management of sickle cell disease in Africa 30 Min Pr B Ranque 80 people
    – Transfusion indication Pr JB Arlet 30 min 80 people
  • Association between erythrocyte microparticles and sickle cell vasculopathy in sub-Saharan Africa: Abdoul Karim DEMBELE, Claudine LAPOUMEROULIE, Mor DIA*, Oumarou TESSOUGUE, Lucile OFFREDO, Dapa DIALLO, Saliou DIOP, Jacques ELION, Yves Colin-Aronovicz, Pierre-Louis THARAUX, Xavier JOUVEN, Marc ROMANA, Brigitte RANQUE*, Caroline LE VAN KIM*, Oral communication at the French Society of Hematology in Paris in March 2019
  • Investigation of risk factors for deterioration of renal function in patients with sickle cell disease in sub-Saharan Africa.
    Prunelle Getten, Youssef Traore, Ismael Kamara, Mourtalla Gueye, Lucile Offredo, Mor Diaw, Saliou Diop, Aissata Tolo, Karim Dembele, Dapa Diallo, Brigitte Ranque, Oral communication at the SNFMI in December 2019
  • Phenotypic differences between sickle cell patients of sub-Saharan origin born in France vs. In Africa; Honsel, B Ranque…, J.B. Arlet, Dorys sickle cell congress. Strasbourg 09/04/2019 200 people
  • Phenotypic differences between sickle cell patients of sub-Saharan origin born in France vs. In Africa ; Honsel, B Ranque…, J.B. Arlet, Congress of the SNFMI national society of internal medicine. June 6, 2019 120 people

2022

Sickle cell disease, the most common genetic disease detected at birth, will soon be screened in all newborns.
Le Monde | 11.15.2022

Between 20,000 and 30,000 people suffer from this condition in France. The High Authority of Health now recommends a systematic neonatal screening.
> Read more

Generalized screening for sickle cell disease : « It’s important that there is this recognition » for patients and « it’s important not to miss patients, » says a doctor.
France Info | 11.18.2022

Professor Jean-Benoît Arlet, a physician at Georges-Pompidou hospital in Paris, reacts to the announcement by the Ministry of Health to extend screening for diseases at birth.
> Read more

2021

TRIBUNE. « We call for sickle cell disease to be the great national cause of 2022 »
Le journal du dimanche | 06.19.2021

On the occasion of World Sickle Cell Day, 45 health professionals, patient associations, national and local elected officials and researchers believe that « it is time to act ». « Sickle cell disease is a forgotten disease, unknown to the general public », they regret.
> Read more

2019

Sickle cell disease, a neglected genetic disease
Le Monde | 06/19/2019

On the occasion of the World Day, focus on an unknown condition that affects 300,000 newborns in Africa every year.
> Read more

2017

Sickle cell disease: success of a gene therapy in the first patient treated
Le Monde | 03/01/2017

Affected by a very severe form of the disease, the teenager treated at the Necker hospital no longer has painful attacks and lives normally without transfusion.
>Read more

Sickle cell disease: « There are two big hopes, bone marrow transplantation and gene therapy »
TV5 Monde | 03/01/2017

With 150 million patients on the planet, it is the first genetic disease in the world. Sickle cell disease mainly affects black and Mediterranean people. Interview with Professor Jean-Benoît Arlet, internist at Georges Pompidou Hospital and member of the NGO Drep.Afrique.

Sickle cell disease: what progress has been made?
RFI | 19/06/2017

Today is World Sickle Cell Day, the most common genetic disease in the world. This hereditary disease, unrecognized, is transmitted to some children only if both parents are carriers of the sickle cell gene. What are the advances in treatment?
> Read more

2016

Jean-Benoît Arlet, a French version of Dr House
Le Monde | 11/21/2016

This physician-researcher specializing in sickle cell disease organizes an annual diagnostic competition that highlights the challenges faced by internists.
> Read more

Contact information

Georges-Pompidou european hospital
> Internal medicine department

20 rue Leblanc
75015 Paris

> Read more

At the Georges-Pompidou european hospital, the reference center for major sickle cell syndromes and other rare pathologies of the red blood cell and erythropoiesis in brief …

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patients followed at least once a year*
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medical consultations*
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day hospitalizations*
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fully hospitalized patients*
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authorized therapeutic education program*
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patients trained in therapeutic education*
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ongoing research projects*
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university courses*
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publications*
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teleconsultation procedures*

* data valid for 2021