EM · Haematology and general medical emergencies

Sickle cell crisis

Also known as Vaso-occlusive crisis · Acute chest syndrome · Splenic sequestration crisis · Aplastic crisis · Sickle cell pain crisis

Sickle cell crisis is the umbrella for the acute, life-threatening events of sickle cell disease (HbS disease): the vaso-occlusive pain crisis, acute chest syndrome, splenic sequestration crisis and aplastic crisis. The Fellowship-critical syndromes are acute chest syndrome — fever with a new pulmonary infiltrate and hypoxia, the leading cause of death in sickle cell disease — and the rapidly fatal childhood splenic sequestration. The management backbone is oxygen (target saturation greater than 95 per cent), cautious isotonic hydration at no more than maintenance once euvolemic, and rapid parenteral opioids (within 30 minutes of triage, reassessed every 15 to 30 minutes). Severe acute chest syndrome with rapid progression is treated with urgent exchange transfusion; acute stroke is treated with exchange transfusion. When red-cell exchange is performed, AABB usual goals are an HbS fraction of not more than 30 per cent and a haematocrit under 30 per cent. Chronic transfusion programmes for stroke prevention keep HbS below 30 per cent immediately prior to the next transfusion, and simple transfusion in non-chronically transfused SCA should not target a haemoglobin above 100 g/L. The reticulocyte count is the diagnostic discriminator: it is high in the vaso-occlusive, sequestration and haemolytic crises but absent in the parvovirus B19 aplastic crisis. ACEM-primary, globally tagged.

Checked against its sources on 2 Sept 2026