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Six haemophilia B patients were studied while undergoing infusion with factor IX concentrate. All were negative for factor IX antigen (IX:AG) and inhibitor to factor IX coagulant activity (IX:C). One patient showed an atypical response pattern, with prolonged survival of IX:C and IX:Ag. This patient remained under prophylactic treatment and more than 1 year later developed an inhibitor to IX:C of clinical significance. Retrospective study revealed that this patient had significantly higher levels of circulating immune complexes than other haemophilia B patients and in vivo formation of immune complexes containing IX:Ag prior to detection of his inhibitor in conventional clotting assays, suggesting long-term persistence of an occult inhibitor. The inhibitor was shown to be an IgG antibody with both kappa and lambda light chains.
C H Miller, and
K H Orstavik, and
M W Hilgartner
January 1988,
Clinical and laboratory haematology,
C H Miller, and
K H Orstavik, and
M W Hilgartner
September 2016,
Haemophilia : the official journal of the World Federation of Hemophilia,
C H Miller, and
K H Orstavik, and
M W Hilgartner
January 2014,
Hamostaseologie,
C H Miller, and
K H Orstavik, and
M W Hilgartner
May 1993,
Lancet (London, England),
C H Miller, and
K H Orstavik, and
M W Hilgartner
January 1975,
Thrombosis research,
C H Miller, and
K H Orstavik, and
M W Hilgartner
July 1997,
Haemophilia : the official journal of the World Federation of Hemophilia,
C H Miller, and
K H Orstavik, and
M W Hilgartner
October 1996,
Haemophilia : the official journal of the World Federation of Hemophilia,
C H Miller, and
K H Orstavik, and
M W Hilgartner
June 1993,
British journal of haematology,
C H Miller, and
K H Orstavik, and
M W Hilgartner
January 2023,
Haemophilia : the official journal of the World Federation of Hemophilia,
C H Miller, and
K H Orstavik, and
M W Hilgartner
May 2022,
Haemophilia : the official journal of the World Federation of Hemophilia,
