HOMOZYGOUS C8 DEFICIENCY REVEALED BY RECU RRENT NEISSERIA-MENINGITIDIS INFECTIONS IN AN ADOLESCENT

Background. - Meningococcal infections associated with late complement component deficiency are rarely severe and usually occur during adole scence and adulthood. We report severe manifestations in a boy in whom the first episode appeared early. Case report. - A 14 year-old gipsy boy was admitted because of a febrile meningococcal meningitis that wa s complicated by a rapidly extensive and necrotic purpura, obnubilatio n and clotting abnormalities without hemodynamic anomalies. The patien t was given symptomatic therapy and a 12-day course of antibiotics tha t resulted in rapid and complete recovery. Medical history of this pat ient showed that he had been admitted at the age of 3 years for a seve re febrile purpura with septic shock and clotting abnormalities follow ed by rapid and complete recovery after symptomatic and antibiotic the rapy. No germ had been then isolated The complement system was studied 3 weeks after the second hospitalization: total hemolytic complement activity could not be detected and C2, C3 and C4 were normal. Examinat ion of the terminal pathway revealed total C8 deficiency. The patient received meningococcal vaccine and was discharged on oral penicillin p rophylaxis. He remained healthy during the ensuing 4 years. Conclusion s. - Meningococcal infections associated with late complement componen t deficiency are generally uncomplicated but they remain potentially s evere. Early screening for this late complement component deficiency s hould be considered after severe clinical manifestations.