Use of prothrombin complex concentrate for prophylaxis of bleeding in acquired factor X deficiency associated with light-chain amyloidosis

Chemotherapy and splenectomy are the most frequently used treatments in cases of systemic light-chain amyloidosis with factor X deficiency; although amyloid deposition is reduced and the main site of its absorption is removed, improvement of factor X levels is not observed in all cases. In order to treat or prevent bleeding events, infusions of fresh-frozen plasma, PCC, activated PCC, recombinant activated factor VII or factor X are suggested options in cases with persistent factor X deficiency5. In our experience, PCC infusion at a dosing regimen of 20 U/kg three times a week in factor X deficiency secondary to amyloidosis was effective and followed by a reduction in the frequency and extent of major bleeding requiring hospitalisation, while a dosing regimen of PCC 20 U/kg twice a week proved to be less effective in preventing severe bleeding.