Acute presentations in treated beta thalassaemia patients

Acute presentations of treated patients

• Fever and Infections
  • all splenctomised patients should have appropriate extra immunisation against pneumococcus. (1)
  • major cause of death in treated patients with thalassaemia (1)
  • mainly caused by gram negative agents such as Klebsiella, E Coli, sometimes salmonella species, with risk of gram positive infection in patients with central lines (1)
  • presents as high fever, and with features of pneumonia, biliary tract infection, meningitis or cerebral abscess and occasionally circulatory collapse. (1)
  • treatment is with prompt broad spectrum antibiotics such as intravenous gentamicin and piptazobactam and vancomycin or teicoplanin in patients with central lines (1)
  • there are also higher incidences of Yersinia enterocolitica infection compared to general population as it thrives in high iron conditions. This can lead to infection in the tonsil or bowel, or can cause septicaemia
  • patient’s presents with fever and abdominal pain sometimes misdiagnosed as acute abdomen. All chelating agents should be stopped until treatment is completed. Antibiotic of choice is ciprofloxacin. (1)
  • suspicion of transfusion related infections such as hepatitis B, hepatitis C and rarely HIV (1)
    
    
    
• Abdominal pain
  • cholelithiasis and biliary colic or obstruction with or without infection
    
    
• Cardiac
  • sudden onset of dysrhythmias or decompensation of ventricular function with fulminant heart failure (1)
  • heart failure can also present as abdominal swelling and pain from hepatic enlargement and ascites. Specialist care should be sought out and treated with diuretics and ACE inhibitors and intensive chelation. (1)
    
    
• Endocrine
  • hypo-hyper glyceamia (1)
    
    
• General treatment
  • these patients may tolerate aneamia less and Hb should be kept at around 12g/dl (1)
    

Reference

• (1) Standards for the Clinical Care of Children and Adults with Thalassaemia in the UK, United Kingdom Thalassaemia Society 2008