Acute chest syndrome in sickle-cell disease
Lancet (London, England)
|January 7, 1984
Summary
Acute chest syndrome (ACS) in sickle-cell disease often presents subtly. Bilateral lung disease on X-ray indicates severe illness, but prompt exchange transfusion can significantly improve outcomes.
Area of Science:
- Hematology
- Pulmonology
- Internal Medicine
Background:
- Acute chest syndrome (ACS) is a common and serious complication in adult patients with sickle-cell disease.
- Early clinical signs and radiological findings of ACS can be non-specific, complicating timely diagnosis and management.
Purpose of the Study:
- To retrospectively analyze the clinical presentation, radiological findings, and outcomes of acute chest syndrome episodes in adult sickle-cell disease patients.
- To identify factors associated with disease severity and treatment response in ACS.
Main Methods:
- Retrospective review of 25 consecutive ACS episodes in 13 adult sickle-cell disease patients.
- Analysis of presenting symptoms, chest signs, chest X-ray findings, laboratory data, and treatment interventions.
Main Results:
- Chest symptoms were present on admission in 23/25 episodes, but abnormal chest signs and X-rays were less frequent initially.
- Bilateral radiological changes were associated with higher pulse rates, prolonged fever, severe hypoxemia, and greater hemoglobin drops.
- Two deaths occurred in patients with bilateral disease and low platelet counts; 11/12 patients improved with exchange transfusion.
Conclusions:
- Pulmonary intravascular sickling is a likely contributor to the clinical presentation of ACS.
- Bilateral radiological involvement signifies a more severe ACS episode requiring aggressive management.
- Exchange transfusion is an effective treatment for severe ACS in sickle-cell disease.
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