Review Note

A 16 year old boy with known sickle-cell disease is brought to the emergency department profoundly unwell. He has a reduced conscious level, temperature 39°C, pulse rate 160 beats/min and blood pressure 70/40 mmg.Investigations: haemoglobin 78 g/L; MCV 68 fL; WCC 17 * 109/L; platelet count 480 × 109/L; reticulocyte count 120 x 109/L; CRP 41 mg/L.Blood film: neutrophilia with toxic granulation; red cell changes consistent with sickle cell disease and hyposplenism. What is the most likely clinical diagnosis?

• Aplastic crisis
• Infectious mononucleosis
• Meningococcal sepsis
• Painful vaso-occlusive crisis 
• Sickle chest syndrome
  

Meningococcal sepsis

Patients with sickle-cell disease have compromised splenic function resulting from microvascular occlusion. This puts them at risk of life-threatening infections from capsulated organisms. Aplastic crises produce a very low haemoglobin with a reticulocytopenia. There is no history of joint pain or shortness of breath to suggest a vasoocclusive painful crisis or sickle chest syndrome.

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