Chronic meningococcemia in a child with a deficiency of the sixth component of complement

Abstract

Chronic meningococcemia represents an uncommon manifestation of meningococcal disease. Microbial and host factors which may predispose to this form of meningococcal disease are not understood. Although acute meningococcal disease is frequently found in patients with terminal complement deficiencies, the relationship of chronic meningococcemia to complement deficiencies is unclear. We present a case report and a review of the literature describing chronic meningococcemia in association with deficiencies of the complement system. A total of eight cases were identified, all of whom were male. Six of the eight patients were children and two of the eight had a previous history of meningococcal disease. This case report, in conjunction with the previously reported cases, suggests an association between complement deficiencies and chronic meningococcemia.

Chronic meningococcemia presenting with recurrent painful rash and poly-arthralgia without fever

“…Search for complement abnormality and immunoglobulin deficiency was performed although chronic meningococcemia occurred more frequently in immunocompetent patients [1,3,5]. In our patient, the levels of classic and alternative complement, as well as lectin pathways and the complement-regulating protein properdin, were normal.…”

Chronic meningococcemia in a 16-year-old boy: a case report

“…The pathophysiology of chronic meningococcemia remains unclear. N. meningitidis of serogroup B seems to be more often involved than other serogroups [5,6]. Some authors state that the relatively reduced virulence of serogroup B may partly explain the chronicity of clinical signs and the reduced inflammatory reponse [6].…”

Endotoxin release and cytokine production in acute and chronic meningococcaemia

“…Host factors rather than determinants of bacterial virulence are usually held responsible for the relatively benign clinical course of chronic meningococcaemia [3]. Indeed, a number of these patients were proven to have a late complement component deficiency [14]. However, in a series of 15 patients with chronic meningococcaemia, total plasma IgG levels were somewhat lower, but the vast majority of patients had a normal humoral immune system [15].…”