Fulminant Pneumococcemia and Sickle Cell Anemia

Four children with sickle cell hemoglobinopathies died of fulminant pneumococcal infection within several hours of the onset of symptoms. Two of the children had evidence suggestive of intravascular coagulation. It is proposed that sickle cell hemoglobinopathies are host factors, similar to the postsplenectomy state, that predispose the patient to fulminant pneumococcal infection. Children with sickle cell hemoglobinopathies and acute febrile illness, especially those less than 6 years of age, should receive benzyl penicillin G. Oxygen, sodium bicarbonate administered intravenously, and possibly heparin sodium should be given at the earliest sign of altered sensorium, drop in blood pressure, or the appearance of petechiae. In addition, our data support the previously reported association of single or recurrent attacks of nonfulminant pneumococcal meningitis with hemoglobin SS disease. Hemoglobin SS disease should be added to the list of causes of recurrent bacterial meningitis.