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Studies on Christmas Disease: Investigation and Treatment of a Familial Acquired Inhibitor of Factor IX
Author(s) -
George J. N.,
Miller G. M.,
Breckenridge R. T.
Publication year - 1971
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1971.tb03445.x
Subject(s) - factor ix , disease , medicine , antibody , factor ixa , immunology , factor x , platelet , thrombin
S ummary . A family is reported in which three of four members with Christmas disease have developed an inhibitor to factor IX. Studies of this inhibitor have demonstrated that it is an IgG antibody directed against the activated form of factor IX (IXa). There was no immunologically detectable factor‐IX‐like material in the affected family member without an inhibitor. This is consistent with previous postulates that inhibitors to factor IX would only develop in patients with Christmas disease who lack the factor‐IX antigen. One member of this family who had developed an inhibitor has been successfully managed through two dental procedures after transfusion with a factor‐IX concentrate.