Prekallikrein (Fletcher factor) deficiency in clinical disease states

Abstract

Plasma prekallikrein activity was measured in a clotting test in 315 consecutive patients undergoing coagulation profiles during the seven month period between January 1, 1979, and August 3, 1979, using substrate from one of our patients with severe Fletcher factor deficiency. Among the 68 patients with significantly reduced Fletcher factor activity (< 0. 50 U/ml), 20 had chronic liver disease, 13 had disseminated intravascular coagulation (DIC), 11 had sepsis, 4 had chronic renal failure, 4 had Vitamin K deficiency, 3 had nonspecific blocking inhibitors, 3 had dilutional product replacement, 2 had thrombotic phenomena, 1 had Fletcher trait, and 7 were of miscellaneous etiology. Partial thromboplastin time (APTT) was prolonged in 42 of the 68. The most commonly associated coagulation abnormality in patients with prekallikrein deficiency was factor VII deficiency, observed in 39 suggesting a link of the intrinsic to the extrinsic coagulation pathway. There was no significant difference in Fletcher factor activity between males and females. Comparison of our findings with studies in the literature is undertaken, with speculation into the underlying pathogenetic mechanism involved in Fletcher factor deficiency in each disease state.