Introduccióna asplenia congénita es infrecuente, su incidencia exacta se desconoce, ya que es común-mente sub-diagnosticada [1][2][3] . Estudios en neonatos con malformación cardíaca describen la asociación con asplenia hasta en 3% 4 . El diagnóstico de asplenia congénita es difícil y requiere un alto índi-ce de sospecha ya que su reconocimiento precoz puede cambiar la morbi-mortalidad asociada esta condición.La presencia de una función esplénica reducida se ve reflejada en una disminución del aclaramiento de antígenos, tanto extracelulares como intracelulares, en una disminución de la respuesta a nuevos antígenos (lipopolisacáridos), en la alteración de la fagocitosis bacteriana y una disminución de los niveles de properdina y tuftsina 5 . Asplenia congénita y purpura fulminans neumocóccico en paciente pediátrico. Reporte de caso con necropsia y revisión del tema Katalina Bertrán S., Alejandro Donoso F., Pablo Cruces R., Franco Díaz R. y Daniela Arriagada S.Congenital asplenia and pneumococcal purpura fulminans in a pediatric patient.Case report with pathological findings and review 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 old girl, with mitral stenosis and recurrent pneumonia 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/l). 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.Key words: Asplenia, Streptococcus pneumoniae, pneumococcal sepsis, purpura fulminans, HowellJolly bodies.Palabras clave: Asplenia, Streptococcus pneumoniae, sepsis neumocóccica, purpura fulminans, corpús-culos de Howell-Jolly.
LPurpura fulminans (PF) es una patología poco frecuente, de extrema gravedad y alta mortalidad. Se caracteriza fundamentalmente por la repenti...