The role of variant alleles of the mannose-binding lectin in the inhibitor development in severe hemophilia 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.
Source: Thrombosis Research - Category: Hematology Authors: Tags: Full Length Article Source Type: research