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3718 Cervical Artery Lesions in Adults with Sickle Cell Disease : Are They Disease-Related ?

Program: Oral and Poster Abstracts
Session: 114. Hemoglobinopathies, Excluding Thalassemia: Clinical and Epidemiological: Poster III
Hematology Disease Topics & Pathways:
Research, Clinical Practice (Health Services and Quality)
Monday, December 12, 2022, 6:00 PM-8:00 PM

Nour Belkeziz1*, Marie-Pierre Gobin-Metteil2*, Corentin Provost1*, Suella Martino, MD PhD3*, Myriam Edjlali4*, Nicolas Mele1*, Benjamin Maïer2*, Anne Blanchet-Deverly5*, Jean-Benoit Arlet6*, Laure Joseph, MD7*, Pablo Bartolucci, MD, PhD8* and David Calvet, MD, PhD2*

1Centre Hospitalier Sainte-Anne, Paris, France
2Centre Hospitalier Sainte-Anne, Paris, FRA
3APHP Hôpital Henri Mondor, Creteil, France, Creteil, France
5CHU de Guadeloupe, Poite à Pitre, France
6Hopital Europeen Georges Pompidou, Paris, FRA
7Biotherapy Department, French National Sickle Cell Referral Center, Hôpital Necker, Assistance-Publique Hôpitaux de Paris, Paris, DC, France
8Université Paris-Est Créteil, INSERM, IMRB, Laboratory of excellence LABEX GRex, Créteil, France


Stroke is one of the main causes of morbidity and mortality in sickle cell disease (SCD) patients, both children and adults. Most cases of stroke are ischemic and associated with a specific narrowing of large intracranial arteries called “SCD-vasculopathy”. Cervical artery lesions were also reported as potentially responsible for strokes in children but very little is known in adults. We aimed to describe cervical artery lesions in SCD adults and assess the associated risk of stroke.

Design and Methods

We included consecutive SCD adult patients referred to our hospital for a neurological assessment. Cervical arteries were analyzed using a standardized ultrasound Doppler protocol. Lesions were dichotomized as (i) segmental: dysplastic, atherosclerotic or sinuosity or (ii) non segmental (occlusion or near occlusion). Brain MRI was used to assess brain lesions.


Among 216 patients, a lesion of cervical arteries independent from an intracranial vasculopathy was present in 101 patients (47%, 95% CI : 40-53). Among those 101 patients, the lesion involved at least one carotid artery in 92 patients, at least one vertebral artery in 32, and both in 23. The were mainly segmental lesions : dysplastic lesion in 45/101, and sinuosities in 55/101. An occlusion or sub-occlusion of the carotid artery was identified in 6.

Among 81 patients with extracranial internal carotid lesions and no ipsilateral intracranial vasculopathy, 39 had brain lesions on MRI, which were territorial infarcts in 21. Five patients had an ischemic stroke during a 47 months follow-up including 2 due to the initial cervical artery lesion. One of them is illustrated in Figure 1.


Cervical artery lesions are common in SCD patients. They may be responsible for cerebrovascular complications such as brain lesions and stroke. The need for a systematic screening and prevention strategy will have to be assessed.

Disclosures: Bartolucci: Bluebird bio: Consultancy, Research Funding; Emmaus: Consultancy, Research Funding; Fabre Foundation: Research Funding; Innovhem: Other: Co-founder; Hemanext Inc: Consultancy; JazzPharma: Consultancy; Global Blood Therapeutics: Consultancy, Research Funding; Novartis: Consultancy, Research Funding; Agios: Consultancy, Research Funding; Roche: Consultancy; Addmedica: Consultancy, Research Funding.

*signifies non-member of ASH