Christmas Disease Associated with Factor VII Deficiency

The Function of Factor VII

“…If it is accepted that, in the absence of deficiencies in other coagulation factors, the one-stage prothrombin time accurately reflects the level of Factor VII in the blood, then it seems necessary to postulate that cases in the third group must have had some other deficiency beyond that of Factor VII, for in this group minor prolongatioiis in the prothrombin time were associated with major clotting abnormalities. Indeed, Bell and Alton (1955) suggested that their three cases which fall into this group (Cases 25, 30, 31) may have suffered from combined Factor-VII and Christmas-factor deficiency. Thc association of Factor-VII deficiency with a deficiency of some other coagulation factor could well be the explanation of the findings in the other cases in this group.…”

Familial Haemophilia and Factor Vii Deficiency

“…Cases of combined deficiencies of different clotting factors are extremely rare and up till now there have been reported combinations of haemophilia and factor V deficiency (Koller, 1954), haemophilia and Christmas disease (Soulier and Larrieu, 1953), Christmas disease and factor VII deficiency (Bell and Alton, 1955;Stein and Abrahams, 1956; de Vries, Kettenborg, and van der Pol, 1955), but not of haemophilia and factor VII deficiency.…”

Williams trait. Human kininogen deficiency with diminished levels of plasminogen proactivator and prekallikrein associated with abnormalities of the Hageman factor-dependent pathways.

“…7). The (46) and Factor V and VIII deficiency (47). Alternatively, functional absence of an inhibitor might lead to accelerated catabolism of these proteins, as observed with C3b inactivator deficiency (48).…”