Background: Anaemic crises in paediatric patients with sickle cell anaemia are major causes of morbidity and mortality. Some children admitted to hospitals' emergency rooms or paediatric wards of the hospitals with severe anaemia die before blood transfusion. Aims and Methods: A total of 108 episodes of anaemic crises were prospectively evaluated in 108 patients with sickle cell anaemia attending the paediatric sickle cell clinic of the University of Nigeria Teaching Hospital, Enugu, Nigeria. Results: Hyper-haemolytic crises were the commonest types of anaemic crises in the patients studied. The mean haemoglobin concentration of 44.66 (SD 6.42) g/l in crises was significantly lower than the mean steady state level of 78.69 (SD 8.50) g/l. Reticulocytes, unconjugated serum bilirubin concentrations, and the presence of nucleated red blood cells were also increased. About 4.6% of patients were not jaundiced at presentation even though they were profoundly anaemic. Their haematological indices gradually returned to normal without marked changes in their serum bilirubin concentrations. These patients were probably in the early recovery phase of aplastic crises. The classical presentation of acute splenic sequestration crisis with a rapidly enlarging spleen and hypotension was not observed. This was probably because of its precipitate nature and accompanying circulatory collapse, which carried a high mortality rate. Minor forms of sequestration crises may have occurred in the liver and spleen. Conclusions: Malaria appeared to have played a role in precipitating some of the hyper-haemolytic episodes. Further studies to elucidate this role are required so that appropriate recommendations regarding malaria prophylaxis can be made in patients with sickle cell anaemia.

Anemia is a major cause of morbidity and mortality among patients with sickle cell anemia. In this study, 108 episodes of severe anemia were prospectively evaluated in 108 patients with hemoglobin SS disease attending the pediatric sickle cell clinic of the University of Nigeria Teaching Hospital, Enugu, Nigeria. Young children between the ages of 2 and 4 years were found to be at the greatest risk of developing anemic crises (severe anemia). There was a gradual but progressive decline in the incidence of severe anemia in the age range 8-16 years old. Upper respiratory tract infections are the most commonly associated infections in patients with severe anemia. Others included malaria, septicemia, urinary tract infection, acute chest syndrome, and osteomyelitis. Their role in precipitating episodes of severe anemia among the patients studied could not be fully evaluated. Pallor, jaundice, and fever were the most commonly encountered symptoms in patients with severe anemia on admission. About half of the parents/guardians failed to notice severe anemia among the patients studied, perhaps due to the dark color of the African skin. Caregivers need to be educated on how to recognize anemia among patients with sickle cell anemia when they develop febrile episodes. Am. J. Hematol. 72:185-191, 2003.