Pulmonary complications

Acute and chronic pulmonary complications are the leading cause of death in older patients. The acute chest syndrome is characterized by hypoxia, tachypnoea, fever, chest pain and pulmonary infiltrate on chest radiographs (Figure 7.4c). Acute chest syndrome often follows a painful event, particularly in adults (Table 7.3). The pathogenesis of acute chest syndrome involves vaso-occlusion, infection or both. Infections due to Mycoplasma,

Acute Chest Syndrome
Table 7.3 Presenting symptoms of acute chest syndrome.


Children (%)

Adults (%)




Shortness of breath



Chest pain



Extremity pain



Rib pain



Adults are more likely than children to have pain preceding the onset of pulmonary symptoms.

Adults are more likely than children to have pain preceding the onset of pulmonary symptoms.

Chlamydia, Legionella, pneumococcus, H. influenzae and viruses are more likely in children. Fat-laden pulmonary macrophages in the airways due to fat embolization from the bone marrow are present in one-half of the cases. Hypoxia due to acute chest syndrome can result in widespread sickling and vaso-occlusion, with risk of multiorgan failure. Patients should receive supplemental oxygen, incentive spirometry and antibiotic therapy directed towards the common organisms. One commonly used regimen consists of cefuroxime and erythromycin, although antibiotics should be guided by local experience. Most patients have a bronchoreactive component and should receive bron-chodilator therapy. Recent data suggest that early transfusion may prevent the progression of pneumonia. Urgent blood transfusion is always required for persistent hypoxia or worsening lung consolidation. Partial exchange transfusion and mechanical ventilation is sometimes needed in rapidly progressive cases. Nitric oxide and steroids may be beneficial in life-threatening cases.

Chronic pulmonary problems seen in SCD are restrictive and obstructive lung disease, hypoxaemia and pulmonary hypertension. Chronic complications are more frequent in patients with a history of acute chest syndrome. The prognosis for severe pulmonary hypertension is poor and no satisfactory management is available. Hydroxyurea, regular transfusions, vasodilators, anticoagulation and oxygen inhalation have been tried in some patients. Early treatment with transfusion therapy is being evaluated in asymptomatic patients with early pulmonary hypertension.

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