What question did this study set out to answer?

To review the evolving understanding of sickle cell anaemia pathophysiology and summarize developments in therapeutic options targeting vaso-occlusion and vascular biology.

January 21, 2019European Journal Of HaematologyOpen Access

Insight into the complex pathophysiology of sickle cell anaemia and possible treatment

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Authors

APAndrea PiccinUniversity of Trento CMC. MurphyCork University Hospital EEElva EakinsIrish Blood Transfusion Service

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Implication

Review outlines multifaceted mechanisms of vaso-occlusion and endothelial damage in sickle cell anaemia, highlighting targets for novel therapeutics.

Key Points

To review the evolving understanding of sickle cell anaemia pathophysiology and summarize developments in therapeutic options targeting vaso-occlusion and vascular biology.
Synthesized molecular and vascular evidence on sickled hemoglobin dynamics, red cell membrane disruption, and endothelial dysfunction.
Evaluated established FDA-approved treatments alongside recent clinical trial advances for novel disease-modifying agents.
Sickle hemoglobin polymerizes upon deoxygenation, causing recurrent cycles of sickling, membrane breakdown, hemolysis, and vaso-occlusion.
Vascular injury is driven by leukocyte adhesion, microparticle release, and the depletion of endogenous vascular protectants including nitric oxide, protein C, and protein S.
Hydroxyurea and L-glutamine remain cornerstone therapies to suppress polymerization, while multiple targeted drugs undergoing randomized clinical trials show potential to address downstream vascular damage.

Cite This Study

Piccin et al. (2019) studied this question.

synapsesocial.com/papers/6a6fb73f31a3df82432809fb https://doi.org/10.1111/ejh.13212

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Implication

Review outlines multifaceted mechanisms of vaso-occlusion and endothelial damage in sickle cell anaemia, highlighting targets for novel therapeutics.

Insight into the complex pathophysiology of sickle cell anaemia and possible treatment

Discussion

Member takes

Implication

Key Points

Cite This Study

Insight into the complex pathophysiology of sickle cell anaemia and possible treatment

Discussion

Member takes

Implication

Key Points

Cite This Study

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