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Absent or defective splenic function is associated with a high risk of fulminant bacterial infections, especially due to encapsulated bacteria. Not knowing this condition may delay medical treatment. Streptococcus pneumoniae is the leading cause of sepsis in these patients. Asplenic patients are at high risk for septic shock and eventually purpura fulminans, a life-threatening condition. We report the case of a 3 years oíd girl, with mitral stenosis and recurrent pneumonía that was admitted due to fever but in the next few hours presented hypotension, purpura and livedo reticularis. Laboratory test showed leucopenia (3.400/mm(3)), bandemia (43% of immature forms), thrombocytopenia, hypoprothombinemia and severe lactic acidosis (ph: 7.0 and lactic acid 11 mmol/1). The patient developed septic shock and multiorganic failure. Mechanical ventilation, volume resuscitation, vasoactive drugs and antibiotic therapy was initiated. Ultrasound was performed on the second day, demostrating asplenia. Peripheral blood smear showed Howell-Jolly bodies. Patient had a positive blood culture for penicillin-resistant Streptococcus pneumoniae (serotype 19F). Patient died of intracerebral hemorrhage after 8 days of admission. Necropsy confirmed asplenia and bilateral suprarenal haemorrhage. Absence of spleen can lead to life threatening infections, it is important to recognize it because vaccination and antibiotic prophylaxis can provide life-saving protection. This case provides a reminder to pursue asplenia as a potential underlying mechanism for invasive bacterial infection in children.

The neonatal sepsis by Streptococcus pneumoniae is a clinical manifestation well know, but very often reported. It's role as an etiologic agent is limited to sporadic references in the medical literature, since 1972 only about fifty cases of septicaemia and/or meningitis have been reported. In the Instituto Nacional de Perinatología, from a whole of 560 confirmed cases of septicaemia in the newborn within the last 6 years, only one has been documented as systemic infections by S. pneumoniae. This was a 32.2 gestational aged male patient, with antecedent of maternal chorioamnionitis. This patient presented early-onset sepsis and died during the first 12 hours of life. Presence of S. pneumoniae was found in the blood culture. The type of clinical presentation and its correlation to the perinatal infection is discussed.