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Acute Chest Syndrome (Sickle Cell)
🔥 Acute Chest Syndrome (ACS) is the leading cause of mortality in sickle cell disease. Most present with severe bone pain followed by respiratory compromise. 🚨 Urgent haematology consult is essential.
🩺 About
- Occurs in ~50% of people with SCD, especially HbSS or HbS-ß0 thalassemia.
- Often follows an acute pain crisis (VOC).
- Triggers include infection, fat embolism, and pulmonary infarction.
⚡ Aetiology
- Chest infection / pneumonia
- Fat embolism from necrotic bone marrow
- Pulmonary infarction
📊 Clinical
- New rib, sternal, or vertebral pain
- Bilateral chest signs (crepitations)
- Tachypnoea >20/min
- Hypoxia with falling Hb
- Fever
🧪 Investigations
- FBC: Anaemia, leukocytosis, ↑ reticulocytes
- LFT: Elevated bilirubin
- CXR: Bilateral infiltrates (hallmark of ACS)
- Sickle solubility test: Positive
- Electrophoresis: HbS ± HbC/HbD, usually no HbA (except in HbS/ß+ thalassaemia)
🧾 Other Complications
- Pneumococcal septicaemia
- Splenic sequestration
- Parvovirus B19 → marrow aplasia
- Folate deficiency
🛠️ Management
- ABC approach, high-dependency/ICU bed
- Oxygen support (consider CPAP or BiPAP)
- Aggressive analgesia for VOC
- Exchange transfusion: reduce HbS <20%
- IV antibiotics (cover atypicals + pneumococcus)
- Prevent recurrence: hydroxycarbamide (↑ HbF) or regular transfusions
🖼️ Graphic
📚 References
💡 Clinical Pearl: Any sickle cell patient with new chest pain + infiltrates on CXR = ACS until proven otherwise. Early exchange transfusion saves lives.
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