Low fetal hemoglobin percentage is associated with silent brain lesions in adults with homozygous sickle cell disease

Author:

Calvet David1,Tuilier Titien2,Mélé Nicolas1,Turc Guillaume1,Habibi Anoosha3,Abdallah Nassim Ait3,Majhadi Loubna4,Hemery François5,Edjlali Myriam6,Galacteros Frédéric3,Bartolucci Pablo3

Affiliation:

1. Service de Neurologie, Centre Hospitalier Sainte-Anne, Université Paris Descartes, Département Hospitalo-Universitaire (DHU) Neurovasc Sorbonne Paris Cité, INSERM U894, Paris, France;

2. Service de Neuroradiologie, Hôpital Henri-Mondor, Université Paris-Est Créteil (UPEC), Créteil, France;

3. Sickle Cell Referral Center, Service de Médecine Interne, Hôpital Henri-Mondor (Assistance Publique–Hôpitaux de Paris [APHP]), UPEC, INSERM U895, Team 2, Laboratoire d’Excellence GRex, Créteil, France;

4. Service de Neurologie, Hôpital Henri-Mondor, UPEC, Créteil, France;

5. Département Informatique Médicale, Hôpital Henri-Mondor (APHP), Créteil, France; and

6. Service de Neuroradiologie, Centre Hospitalier Sainte-Anne, Université Paris Descartes, DHU Neurovasc Sorbonne Paris Cité, INSERM U894, Paris, France

Abstract

Key Points Low %HbF is independently associated with silent WMCs on brain imaging in adults with SCD. Our results highlight the potential use of therapeutic strategies inducing HbF expression in SCD patients with silent white matter changes.

Publisher

American Society of Hematology

Subject

Hematology

Reference49 articles.

1. Mortality in children and adolescents with sickle cell disease;Leikin;Pediatrics,1989

2. Mortality in sickle cell disease. Life expectancy and risk factors for early death;Platt;N Engl J Med,1994

3. Cerebrovascular accidents in sickle cell disease: rates and risk factors;Ohene-Frempong;Blood,1998

4. Pathophysiology and treatment of stroke in sickle-cell disease: present and future;Switzer;Lancet Neurol,2006

5. Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography;Adams;N Engl J Med,1998

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