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Acute chest syndrome in sickle cell anaemia

Dr Jan Frank Gerstenmaier and Dr Yuranga Weerakkody et al.

Acute chest syndrome in sickle cell anaemia is a leading thoracic complication as well as leading cause of mortality in those affected by sickle cell disease 3. 

For a general discussion of sickle cell disease, please refer to the article : sickle cell disease. 

Clinical presentation

Patient's may present with acute fever, cough and wheezing on a background of established sickle cell disease. 

Pathology

The pathological basis is thought to be from pulmonary microvascular occlusion and haemolysis from distorted sickle cells as a result of a several factors including exposure to low O2 tension.

Radiographic features

Plain film

Typically seen as segmental / sub segmental atelectasis +/- consolidation with a lower lobe predilection +/- pleural effusion. 

A chest radiograph may also show other sequelae from sickle cell disease such as bone infarcts, rib enlargement and cardiomegaly (from anaemia).  

HRCT

May show a mosaic perfusion pattern +/- a pleural effusion.

Differential diagnosis

General imaging differential considerations include

See also

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