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Table 104.2

Other Acute Complications of Sickle Cell Disease

ComplicationClinical features and management
Acute chest syndrome

Chest symptoms or signs with a new infiltrate on chest X-ray.

It is impossible to distinguish vaso-occlusive infarction from pneumonia with certainty and you should assume that both are present.

Arterial blood gases are a useful tool for assessing severity and should be done if SaO2 is <94% on air or >3% below baseline.

Treat with antibiotics and oxygen to maintain SaO2 >94%.

If PaO2<9 kPa on air, intervention with transfusion and/or invasive respiratory support should be considered. In this situation haematology and high dependency teams should be consulted.

Stroke

Acute neurological symptoms may indicate infarctive or haemorrhagic stroke, both of which are common in patients with sickle cell disease.

Arrange urgent CT.

Exchange transfusion may be required: seek advice from a haematologist.

Priapism

Painful and persistent penile erection.

If not adequately treated, it may result in permanent erectile failure.

Initial treatment includes exercise, encourage urination, fluid replacement and pain relief. Oral etilefrine may be of help.

If >1h duration and no response to initial management, contact urologist for consideration of penile aspiration.

Splenic sequestrationCommon in children. Rapid enlargement of spleen and fall in haemoglobin. Treated supportively with blood transfusion.
Aplastic crisisAcute anaemia due to parvovirus B19 infection. Characterized by low reticulocyte count. Treated supportively with blood transfusion.