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Endgames Case Report

Breathlessness and chest pain in a patient with sickle cell disease

BMJ 2011; 343 doi: (Published 20 July 2011) Cite this as: BMJ 2011;343:d3797
  1. Harpreet Ranu, specialist respiratory registrar1,
  2. Hubertus Buyck, consultant haematologist2,
  3. Fenella Willis, consultant haematologist2,
  4. Brendan Madden, professor of cardiothoracic medicine1
  1. 1Department of Cardiothoracic Medicine, St George’s Hospital, London SW17 0QT, UK
  2. 2Department of Haematology, St George’s Hospital
  1. Correspondence to: H Ranu harpreetranu{at}

A 47 year old woman with sickle cell disease presented to the accident and emergency department with shortness of breath and chest pain. She had been admitted many times in the past with similar symptoms and had needed treatment, including simple and exchange blood transfusions. She had developed side effects after different forms of iron chelation treatment in the past.

On admission her oxygen saturations were 93% on room air (decreasing to 82% on walking to the toilet), systemic blood pressure was 90/50 mm Hg, and heart rate was 100 beats/min. She had a low grade fever. Her jugular venous pressure was raised, with a gallop rhythm and a loud second heart sound. Breath sounds were quiet and she found it painful to breathe in. Her haemoglobin was 68 g/L (reference range 115-150), with haemoglobin S 55.8%, white cell count was 18×109/L (4-11), and serum ferritin was 26 964 pmol/L (27-337). Chest radiography showed basal alveolar shadowing.


  • 1 What is the differential diagnosis in this patient?

  • 2 How should this patient be managed in the accident and emergency department?

  • 3 What complications are associated with sickle cell disease?

  • 4 What further diagnostic tests would you arrange once the patient has been stabilised?


1 What is the differential diagnosis in this patient?

Short answer

The differential diagnosis includes an acute chest syndrome, a pulmonary embolism including a fat embolus, and pulmonary infection.

Long answer

Acute chest syndrome is one of the most common reasons for hospital admission in patients with sickle cell disease.1 It is defined by the presence of a new pulmonary infiltrate consistent with alveolar consolidation and is often accompanied by cough, chest pain, wheeze, fever, and an increased respiratory rate.2 It is commonly precipitated by community acquired infection, with an infectious cause being identified in more than half of patients admitted with the syndrome, most often atypical bacteria …

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