ImmunoSensation - the immune sensory system

Thromb Res. 2019 May 9.

The role of variant alleles of the mannose-binding lectin in the inhibitor development in severe hemophilia A.

Ulrich-Merzenich G, Hausen A, Zeitler H, Goldmann G, Oldenburg J, Pavlova A

The administration of FVIII leads to inhibitors in up to 30% of patients with hemophilia A (HA), the most severe treatment complication. FVIII-mannosylation fosters the presentation of FVIII to CD4-T-lymphocytes. Mannose as primary ligand for the mannose-binding lectin (MBL) activates the lectin pathway of complement. MBL2 single nucleotide polymorphisms (SNPs) lead to low peripheral MBL concentrations that may hamper the removal of mannosylated FVIII.

PMID: 31141731