Factors XI and XII are low in subjects with liver disease

Abstract

We prospectively measured levels of factors XI and XII in parallel with other coagulation factors in 39 unselected patients with liver disease and in 20 control subjects. Mean levels of factors XI and XII in subjects with liver disease were significantly reduced, being 58% and 61%, respectively, compared with 100% and 94% in controls. Reductions in levels of factors XI and XII were most pronounced in those subjects with low serum albumin. The partial thromboplastin time (APTT) reflected low levels of either factor XI or XII and was most prolonged when both were low, but cause and effect was not demonstrated. Low levels of these factors may explain previous reports of poor response of APTT to infusions of prothrombin complex concentrates. Finally, these low levels strongly suggest that factors XI and XII are produced in the liver.

The investigation of a prolonged APTT with specific clotting factor assays is unnecessary if an APTT with Actin FS is normal

“…Nearly three quarters of the abnormal results were a deficiency in FXI ranging from 1 IU/dl to 66 IU/dl. Such a reduction does not correlate with or predict the bleeding risk, and acquired FXI deficiency has been described in common disorders including liver disease (Walker et al. , 1983) and systemic lupus erythematosus (Vercellotti & Mosher, 1982).…”

Factor XI: A Review of Its Biochemistry and Deficiency

“…As presented in the preceding section, hepatic maturity appears to correlate well with Factor XI levels in the newborn. Several studies 9,16,18 have demonstrated reductions in Factor XI levels in severe hepatic disease, with levels as low as 30 to 50 U/dl in advanced hepatic cirrhosis. In one study, 18 plasma Factor XI levels paralleled serum albumin concentrations.…”

“…Several studies 9,16,18 have demonstrated reductions in Factor XI levels in severe hepatic disease, with levels as low as 30 to 50 U/dl in advanced hepatic cirrhosis. In one study, 18 plasma Factor XI levels paralleled serum albumin concentrations. One patient undergoing liver transplantation received a liver from a donor who was known to have had a plasma Factor XI level of 26 U/dl; following successful transplantation, the recipient's plasma Factor XI level was 22 U/dl, indicating the lack of meaningful extrahepatic Factor XI production, at least in this one case.…”

How I investigate for bleeding disorders

“…As congenital or acquired deficiencies affecting single factors in the common pathway are very rare compared with vitamin K deficiency and liver disease, factors II, V, VII, VIII, IX, X, XI, and XII are often assessed when there is a history of bleeding and both tests are prolonged . While low factor VII can suggest a coagulopathy of liver disease, other factors including factors XI and XII are also low in many persons with liver disease …”