TableĀ 1

Common clinical presentations of SCD

Clinical presentationSymptomsLaboratory findingsTreatment
Painful crisisPain, localised swelling, feverLow Hb, high reticulocyte count, high LDH, high bilirubin, high CRPHydration
Analgesia
Antibiotics
Acute chest syndromeChest pain, fever, hypoxia, cough; may progress from painful crisis elsewhereLow Hb, high reticulocyte count, high LDH, pulmonary infiltrates on CXRRespiratory support, antibiotics, red cell exchange transfusion
Bacterial sepsisPain, localised swelling, feverLow Hb, high reticulocyte count, high LDH, high CRP, positive culturesHydration
Analgesia
Antibiotics
Sequestration crisisPain, severe pallor, hepatomegaly or splenomegalyLow Hb, high reticulocyte countUrgent red cell transfusion, pain relief
Aplastic crisisPallorLow Hb, low reticulocyte count, parvovirus B19 +veUrgent red cell transfusion
Acute ischaemic strokeHemiplegia, altered consciousness, seizuresMRI brain with characteristic findingsUrgent red cell exchange transfusion aim to reduce HbS to <30%
Girdle syndromeAbdominal pain and distension, reduced or absent bowel sounds, pallor, feverAXR may show dilated bowel loops. Low Hb, high reticulocyte count, high CRPNil by mouth, NG tube on free drainage, broad spectrum antibiotics with anaerobic cover, red cell exchange transfusion, surgical review
PriapismPainful, persistent erectionHydration, pain relief, urgent urology review and intervention: red cell exchange transfusion
  • AXR, abdominal x-ray; CRP, C-reactive protein; CXR, chest x-ray; Hb, haemoglobin; HbS, sickle haemoglobin; LDH, lactate dehydrogenase; MRI, magnetic resonance imaging; NG, naso-gastric; SCD, sickle cell disease.