Acute chest syndrome is one of the complications of sickle cell disease.
Pathogenesis: pulmonary infection, pulmonary fat embolism from necrotic bone marrow and/or pulmonary infarction
Clinical features (vary with age):
(1) acute illness
(2) cough
(3) chest pain
(4) fever (>= 38.5°C)
(5) hypoxemia
(6) tachypnea
(7) dyspnea
(8) variable crepitations on auscultation
Findings on imaging studies: segmental pulmonary infiltrate consistent with consolidation
Complications:
(1) progression to acute respiratory failure
(2) multi-organ failure
(3) neurological complications (stroke, posterior reversible leukoencephalopathy, etc)