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Coagulation factor XI (FXI) is a glycoprotein of 160 kDa, which, upon thrombin-mediated activation, activates factor XII or FXI itself, catalyzing the conversion of factor IX (FIX) to activated FIX in the consolidation phase of blood coagulation.[1][1] FXI deficiency is a rare autosomal recessive

Severe factor XI deficiency in the Abruzzo region of Italy is associated to different FXI gene mutations