Acute chest syndrome: sickle cell disease

Area of Science:

• Hematology
• Pulmonology
• Critical Care Medicine

Background:

• Acute chest syndrome (ACS) is a frequent and life-threatening complication in sickle cell disease (SCD).
• ACS is the leading cause of mortality among individuals with SCD.
• Identifiable triggers for ACS are often elusive, though infection, vaso-occlusive crisis (VOC), and asthma are recognized contributors.

Purpose of the Study:

• To provide a comprehensive review of the pathogenesis, clinical characteristics, complications, and current treatment strategies for ACS in SCD patients.
• To highlight emerging potential etiologies of ACS.
• To identify strategies for improving oxygenation to reduce sickling and lung damage.

Main Methods:

• This study is a comprehensive literature review.
• The review synthesizes existing research on ACS in SCD.
• Focus is placed on identifying new and known triggers, clinical presentations, and therapeutic interventions.

Main Results:

• Infection is the most common identifiable cause of ACS in SCD.
• Vaso-occlusive crisis (VOC) and asthma are significant, though less frequent, triggers.
• New potential etiologies are being investigated to improve management.

Conclusions:

• Effective management of ACS in SCD requires understanding its diverse triggers and clinical spectrum.
• Further research into novel etiologies is crucial for enhancing oxygenation and mitigating lung injury.
• Improving management strategies for ACS can reduce mortality in the SCD population.