Vaso-occlusive crisis in sickle cell disease: a vicious cycle of secondary events

Painful vaso-occlusive crisis (VOC) remains the most common reason for presenting to the Emergency Department and hospitalization in patients with sickle cell disease (SCD). Although two new agents have been approved by the Food and Drug Administration for treating SCD, they both target to reduce th...

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Bibliographic Details
Published in:Journal of translational medicine 2021-09, Vol.19 (1), p.1-397, Article 397
Main Authors: Jang, Tim, Poplawska, Maria, Cimpeanu, Emanuela, Mo, George, Dutta, Dibyendu, Lim, Seah H
Format: Article
Language:English
Subjects:
Development and progression
Downstream events
Drug therapy
Emergency medical care
Endothelium
Erythrocytes
Feedback
Hemoglobin
Hospitalization
Inflammation
Ischemia
Neutrophils
Pain
Review
Secondary vaso-occlusive crisis
Sickle cell disease
Treatment strategies
Vaso-occlusive crisis
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Summary:Painful vaso-occlusive crisis (VOC) remains the most common reason for presenting to the Emergency Department and hospitalization in patients with sickle cell disease (SCD). Although two new agents have been approved by the Food and Drug Administration for treating SCD, they both target to reduce the frequency of VOC. Results from studies investigating various approaches to treat and shorten VOC have so far been generally disappointing. In this paper, we will summarize the complex pathophysiology and downstream events of VOC and discuss the likely reasons for the disappointing results using monotherapy. We will put forward the rationale for exploring some of the currently available agents to either protect erythrocytes un-involved in the hemoglobin polymerization process from sickling induced by the secondary events, or a multipronged combination approach that targets the complex downstream pathways of VOC.
ISSN:1479-5876
1479-5876
DOI:10.1186/s12967-021-03074-z