Mannose binding lectin (MBL) deficiency (ICD-10 D84.8)
Contents
Definition
MBL is an acute phase protein. Some people have persistently low MBL values in the blood, which increases the risk of more serious infections, especially meningococcal disease. MBL deficiency increases the risk of thromboembolism (blood clots). MBL deficiency can probably lead to increased complication risk in systemic connective tissue disorders og vasculitides
Occurrence
About. 5% of the population has MBL deficiency
Clinical significance
MBL deficiency does not cause disease itself, but can increase the severity of infections. Also the course of autoimmune diseases (connective tissue disease and vasculitis) can be affected.
Treatment
No specific treatment. Preventive measures against infection (vaccines, scabies, antibiotics) are relevant. Substitution with immunoglobulins at low blood IgG values.