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Recurrent meningococcal septicaemia and properdin deficiency
N A Cunliffe1, N Snowden, E M Dunbar
1Department of Infectious Diseases and Tropical Medicine, (Monsall Unit) North Manchester General Hospital, Crumpsall, U.K.
Abstract:
A 32-year-old male presented with two episodes of meningococcal septicaemia, each of which was caused by a different serogroup of Neisseria meningitidis. Examination of the alternative pathway of complement revealed the rare X-linked disorder properdin deficiency (PD). Meningococcal Infection in complement deficiency states is discussed and the unusual features of this case are highlighted.
Insights
A patient with recurrent meningococcal septicaemia was diagnosed with properdin deficiency (PD), a rare complement disorder. This case highlights the importance of investigating complement pathways in severe meningococcal infections.
Area of Science:
- Immunology
- Infectious Diseases
- Genetics
Background:
- Recurrent meningococcal septicaemia can indicate underlying immune deficiencies.
- The complement system, particularly the alternative pathway, is crucial for defense against Neisseria meningitidis.
Observation:
- A 32-year-old male experienced two distinct episodes of meningococcal septicaemia caused by different Neisseria meningitidis serogroups.
- Diagnostic workup identified a deficiency in properdin, a key protein in the complement alternative pathway.
Findings:
- The patient was diagnosed with X-linked properdin deficiency (PD).
- This deficiency significantly impairs the alternative complement pathway's ability to combat Neisseria meningitidis infections.
Implications:
- Properdin deficiency is a rare but serious cause of severe meningococcal disease.
- Early diagnosis and management of complement deficiencies are vital for preventing recurrent invasive bacterial infections.