Pneumococcal sepsis and meningitis in adults with sickle cell disease

Patients with sickle cell disease are predisposed to infection caused by Streptococcus pneumoniae. However, there has been only one published case of bacteremic pneumococcal meningitis in an adult with sickle cell anemia. We report here the cases of six adults with sickle cell disease, pneumococcal sepsis, and meningitis. Five patients were male and one was female. Their ages ranged from 18 to 34 years (mean, 25.7 years). Five patients had the SS and one had the SC hemoglobin phenotype. Only one patient had received pneumococcal vaccine (14 valent). This vaccine did not protect against the pneumococcal serotype causing his infection. All patients had high fever (mean, 39.8 degrees C [103.7 degrees F]) on admission; five had generalized weakness and four had neck stiffness. Leukocyte counts were greater than 30,000/mm3 in all patients. Streptococcus pneumoniae was isolated from the blood and the cerebrospinal fluid in all patients. The cerebrospinal fluid showed pleocytosis in six patients, an elevated protein level in five, and hypoglycorrhachia in two. Complications included renal failure in four patients, disseminated intravascular coagulation in one, and seizures in another. Two patients died. Pneumococcal sepsis and meningitis are uncommon in adults with sickle cell disease, but they carry a high morbidity and mortality. Wider use of the new 23-valent polysaccharide vaccine in these patients is recommended.