From the Guidelines
Acute chest syndrome in patients with sickle cell disease should be diagnosed using a combination of clinical evaluation, chest X-ray, complete blood count, reticulocyte count, blood cultures, and arterial blood gas analysis, as recommended by the most recent guidelines 1. The diagnosis of acute chest syndrome (ACS) in patients with sickle cell disease (SCD) is crucial to prevent morbidity and mortality. The clinical presentation of ACS can vary, but common symptoms include chest pain, shortness of breath, fever, and arm and leg pain.
Key Diagnostic Examinations
- Chest X-ray to evaluate for new densities or infiltrates
- Complete blood count to assess for anemia, leukocytosis, or thrombocytopenia
- Reticulocyte count to evaluate bone marrow response
- Blood cultures to identify potential bacterial pathogens
- Arterial blood gas analysis to assess oxygenation and ventilation These examinations are essential to confirm the diagnosis of ACS and to guide management decisions, as outlined in the American College of Cardiology/American Heart Association joint committee on clinical practice guidelines 1.
Importance of Prompt Evaluation
Prompt evaluation and diagnosis of ACS are critical to prevent complications, such as respiratory failure, and to reduce mortality. The American Society of Hematology guidelines suggest that patients with SCD and moderate ACS may benefit from automated red cell exchange, manual red cell exchange, or simple transfusions 1.
Additional Considerations
In addition to these diagnostic examinations, patients with SCD are at increased risk of deep vein thrombosis and infection, and therefore, thromboprophylaxis and antibiotic prophylaxis should be considered, especially in the perioperative period 1. Regular monitoring of oxygen saturation and early recognition of signs of infection or sepsis are also crucial to prevent complications. Early consultation with hematology and pulmonology specialists is advisable to ensure prompt and appropriate management of ACS in patients with SCD.
From the Research
Diagnostic Examinations for Acute Chest Syndrome in SCD
To diagnose acute chest syndrome in patients with sickle cell disease (SCD), several examinations are utilized. These include:
- Physical exam
- Blood work
- Chest imaging, with chest radiograph considered the gold standard for imaging modality 2 Specific diagnostic criteria include a new infiltrate on pulmonary imaging combined with any of the following:
- Fever > 38.5°C (101.3°F)
- Cough
- Wheezing
- Hypoxemia (PaO2 < 60 mm Hg)
- Tachypnea
- Chest pain 2
Laboratory and Imaging Studies
Additional laboratory and imaging studies may be used to differentiate acute chest syndrome from other diagnoses, including:
- Complete blood count
- Comprehensive metabolic panel
- Brain natriuretic peptide (BNP)
- Lactic acid
- Procalcitonin
- EKG
- Troponin level
- D-dimer
- Chest radiograph 2
Importance of Early Diagnosis
Early recognition and initiation of treatment for acute chest syndrome are crucial to prevent and treat acute respiratory failure and minimize irreversible lung damage 3. High clinical suspicion should be maintained in SCD patients who present with painful crises, as acute chest syndrome can present in a severe form in adults older than 20 years 4.