Sickle Cell Crisis Diagnosis and Management

Primary Clinical Manifestations

Sickle cell crisis presents with acute, severe pain that is diagnosed entirely based on the patient's self-report, without requiring any specific laboratory abnormalities, and commonly affects the back, legs, knees, arms, chest, and abdomen, according to the American College of Physicians 1
Pain location varies by age: fingers in infancy; long bones, sternum, ribs, or back in older children and adults, as reported by the American Academy of Pediatrics 1

Acute chest syndrome occurs in more than 50% of hospitalized patients with vaso-occlusive crisis and is a leading cause of death with up to 13% all-cause mortality, according to the American Thoracic Society 1, 4
Presenting signs of acute chest syndrome include new segmental infiltrate on chest radiograph, lower respiratory tract symptoms, chest pain, and/or hypoxemia, as recommended by the American College of Chest Physicians 4
Any patient presenting with chest pain requires urgent exclusion of acute chest syndrome through chest X-ray, as advised by the American Heart Association 1, 4
Any acute neurologic symptom other than transient mild headache requires urgent stroke evaluation, according to the American Stroke Association 4
Stroke occurs in up to 10% of children with sickle cell disease, as reported by the American Academy of Neurology 4

Chronic hemolytic anemia is present at baseline, with typical hemoglobin levels between 60-90 g/L in severe disease, according to the American Society of Hematology 2, 3
Acute and chronic end-organ damage develops progressively, as reported by the National Institute of Diabetes and Digestive and Kidney Diseases 2, 3
Splenic sequestration crisis is characterized by rapidly enlarging spleen and hemoglobin decrease >2 g/dL below baseline, according to the American College of Emergency Physicians 4
Fever or signs of sepsis require immediate blood cultures and antibiotic initiation, as recommended by the Infectious Diseases Society of America 4