Immunologie in de medische praktijk. XXVIII. Gevoeligheid voor meningokokkenziekte door een familiair tekort aan mannosebindend lectine

- An 18-year-old man was admitted with meningitis due to Neisseria meningitidis. Since both his mother and his grandfather on his mother's side had had meningitis at about the same age, the possibility of familial immunodeficiency was investigated. This revealed a mannose-binding lectin (MBL) deficiency in the serum of the patient, his mother and grandfather, as well as in four unaffected family members, in the absence of any other deficiencies in humoral or cellular host defense. MBL is the key molecule in the lectin pathway of the complement system. These observations in three generations of one family provide additional support for a role of MBL deficiency in susceptibility to meningococcal disease. About 10 of the general population has an MBL deficiency. Moreover, the familial nature of this increased susceptibility indicates the need for adequate information to deficient individuals, their relatives, and physicians in order to enable meningococcal disease to be recognised and treated without delay.