Preprint Review Version 1 This version is not peer-reviewed

Neurovascular Manifestations of Sickle Cell Disease

Marialuisa Zedde
* ,
Micol Quaresima
,
Isabella Capodanno
,
Ilaria Grisendi
,
Federica Assenza
,
Manuela Napoli
,
Claudio Moratti
,
Claudio Pavone
,
Lara Bonacini
,
Giovanna Di Cecco
,
Serena D’Aniello
,
Franco Valzania
,
Francesco Merli
,
Rosario Pascarella
Version 1 : Received: 2 July 2024 / Approved: 3 July 2024 / Online: 3 July 2024 (07:31:09 CEST)

How to cite: Zedde, M.; Quaresima, M.; Capodanno, I.; Grisendi, I.; Assenza, F.; Napoli, M.; Moratti, C.; Pavone, C.; Bonacini, L.; Di Cecco, G.; D’Aniello, S.; Valzania, F.; Merli, F.; Pascarella, R. Neurovascular Manifestations of Sickle Cell Disease. Preprints 2024, 2024070319. https://doi.org/10.20944/preprints202407.0319.v1 Zedde, M.; Quaresima, M.; Capodanno, I.; Grisendi, I.; Assenza, F.; Napoli, M.; Moratti, C.; Pavone, C.; Bonacini, L.; Di Cecco, G.; D’Aniello, S.; Valzania, F.; Merli, F.; Pascarella, R. Neurovascular Manifestations of Sickle Cell Disease. Preprints 2024, 2024070319. https://doi.org/10.20944/preprints202407.0319.v1

Abstract

Sickle cell disease (SCD) is a hereditary blood disorder characterized by abnormal hemoglobin, leading sickle shape of red blood cells. It has several vascular complications and the cerebrovascular ones are among the most frequent and severe both in children and in adults. This review summarizes the main neurovascular manifestations of SCD, including acute stroke, silent cerebral infarction, large vessel diseases (moyamoya arteriopathy and aneurysms) and brain bleeding. Both epidemiology, pathophysiology and treatment issues are addressed and prevention of cerebrovascular events, including silent cerebral infarctions, is particularly relevant in SCD patients, being associated to poor functional outcome and cognitive complaints. Transfusions and hydroxyurea are the main available therapy at the moment, but contraindications, availability and complications might prevent their long term use, particularly in low income countries. The role of Transcranial Doppler in monitoring the patients (mainly children) is analyzed and a practical approach has been selected in order to give the main messages from the actual knowledge for a better management of the SCD patients.

Keywords

sickle cell disease; SCD; anemia; ischemic stroke; bleeding; moyamoya arteriopathy; intracranial aneurysm; subarachnoid hemorrhage; fibromuscular dysplasia; MRI; CT; angiography

Subject

Medicine and Pharmacology, Hematology

Copyright: This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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