FATAL PNEUMOCOCCAL SEPTICEMIA IN HEMOGLOBIN SC DISEASE

We retrospectively examined the medical and autopsy records of seven p reviously unpublished cases of fatal pneumococcal septicemia in childr en with hemoglobin SC disease. The earliest death occurred in a 1-year -old child who had congenital heart disease with cyanosis; the other c hildren were aged 31/2 to 15 years. Only one child had received pneumo coccal vaccine or prophylactic penicillin therapy. All seven children had an acute febrile illness and rapid clinical deterioration despite parenterally administered antibiotic therapy and intensive medical sup port. Erythrocyte pit counts in two patients were 40.3% and 41.7%, res pectively (normal, less than or equal to 3.6%). Autopsy data from five cases showed marked splenic congestion without infarction in five, sp lenomegaly in four, and bilateral adrenal hemorrhage in three. These c ases illustrate that functional asplenia predisposes some children wit h hemoglobin SC disease to the development of fatal septicemia after t he age of 3 years. We conclude that pneumococcal vaccine should be adm inistered to all children with hemoglobin SC disease and that acute fe brile illnesses should be investigated promptly for the possibility of septicemia. The routine use of prophylactic penicillin therapy in inf ants and children with hemoglobin SC disease remains controversial.