Osteomyelitis in sickle cell haemoglobinopathy with elevated fetal haemoglobin

The prevalence rate and characteristics of osteomyelitis in children below 15 years of age were studied in a population characterized by a high prevalence of sickle cell disease (SCD) with elevated fetal haemoglobin (HbF). All children born in our institution routinely undergo a haemoglobinopathy screening test. Osteomyelitis developed in 25 children (16 boys and nine girls) with a mean age of 5.5 years. Fourteen had sickle cell disease (SCD) and 11 had no haemoglobinopathy. The prevalence of osteomyelitis in children with SCD was 69 times higher than in those without (283 vs 4/10(5)). All 14 children with SCD and osteomyelitis also had elevated HbF. Three children had HbS alpha-thalassaemia, with mean HbF 24% and HbS 68.6%. Eleven children had sickle cell anaemia with elevated HbF, mean HbF 31.7% and HbS 66.7%. There was no significant difference in symptoms, signs, leucocyte, neutrophil and band counts, nor in sedimentation rates. Salmonella organisms were significantly more frequently responsible for osteomyelitis in SCD. The presence of elevated HbF in children with SCD should not lead to complacency as they are still at increased risk of developing osteomyelitis.