Inhibitor of Factor VIII in Mild Haemophilia

Beck, Peter; Giddings, J. C.; Bloom, A. L.

doi:10.1111/j.1365-2141.1969.tb01374.x

Cited by 30 publications

(14 citation statements)

References 10 publications

(9 reference statements)

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“…Re-addition of FVIIIC concentrate in vitro again demonstrated incomplete inactivation of FVIIIC activity with some depletion of inhibitory activity (Fig 1). The antibody was thus characterized as transient and of low titre with in vitro studies consistent with type I1 kinetics, findings similar to those described in other cases of mild haemophilia A with inhibitors (Crowell, 19 70;Robboy et al, 1970;Lechner et al, 1973;Beck et al, 1969;Rosenthal, 1957;Strauss, 1969;Shapiro, 1975).…”

Section: Resultssupporting

confidence: 82%

“…While FVIIIC inhibitors occur in from 5% to 10% of patients with haemophilia A (Feinstein, 1983), they are rare in mild and moderate haemophilia. To our knowledge only eight cases have been reported in the literature describing the occurrence of an inhibitor in patients with mild haemophilia A (Crowell, 1970;Robboy et al, 1970;Lechner et al, 1973;Beck et al, 1969;Rosenthal, 1957;Straws, 1969;Shapiro, 1975). Inoneofthesecases(Rosenthal,1957)FVIIIClevels werenot actually measured, and in two others the patients received porcine FVIIIC concentrate (Beck et al, 1969).…”

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confidence: 99%

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Factor VIII antibody in a patient with mild haemophilia

1985

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We present the rare occurrence of an inhibitor of factor VIII procoagulant arising in a patient with mild haemophilia A and rheumatoid arthritis. The inhibitor was transient and behaved like a low titre, type II factor VIII procoagulant inhibitor similar to previously reported cases (Biggs et al, 1972b). In vitro studies confirmed the type II-like interaction of this inhibitor with the factor VIII procoagulant molecule. Factor VIII procoagulant antigen level was equal to the factor VIII procoagulant activity, which excluded dysproteinaemia as the cause. This patient's HLA type has no known association with abnormal immune responsiveness or autoimmune disease, and his clinical course as well as in vitro studies were similar to the eight previously reported cases of factor VIII procoagulant inhibitors arising in mild haemophilia A.

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confidence: 82%

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confidence: 99%

Factor VIII antibody in a patient with mild haemophilia

1985

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“…Such differences may account for the development of inhibitors in mild haemophiliacs (Beck et al, 1969) and for the fact that they develop in only a small proportion of even severely affected patients. It seems possible that this complication may be preventable with greater knowledge of normal and abnormal variants of factor VIII.…”

Section: Factor VIIImentioning

confidence: 99%

Coagulation Factor Variants

1972

Br J Haematol

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“…In our recent paper (Beck et al, 1969) we describe two patients with mild haemophilia who developed inhibitors of factor VIII during replacement therapy to cover major surgery. In both patients the inhibitory activity rapidly waned and they eventually regained their previously mild haemophilic state.…”

Section: Inhibitor Of Factor VIII In Mild Haemophiliamentioning

confidence: 99%

Inhibitor of Factor Viii in Mild Haemophilia

Beck¹,

Giddings²,

Bloom³

1970

Br J Haematol

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In our recent paper (Beck et al, 1969) we describe two patients with mild haemophilia who developed inhibitors of factor VIII during replacement therapy to cover major surgery. In both patients the inhibitory activity rapidly waned and they eventually regained their previously mild haemophilic state. Since 1966, Case I has received cryoprecipitate on eight separate occasions for various traumatic lesions. When tested he has had the normally expected rise of plasma factor VIII and his basal level remained at 8%. In July 1969, however, and since our paper went to press, h s patient was treated for a traumatic haematoma in the forearm muscles. He received two doses of 8 packs of cryoprecipitate, each dose containing approximately 800 ml plasma equivalents (units) of factor VIII. The response to the first dose, given at night, was not assayed but after the second dose his factor-VIII level rose from 8% to only 17%. During the next few days it fell to less than 1% and a weak inhibitor, 0.9 units per ml (Biggs & Bidwell, 195g), appeared in his plasma. The haematoma resolved but at a slower rate than would be expected in a normally responsive patient. Six weeks later his factor VIII was 1% but the inhibitor was no longer detectable. Our experience indicates that an inhibitor developing in a mild haemophiliac can recur with subsequent treatment: a situation analogous to that seen in severely affected patients. It is not clear why the inhibitor should have recurred following the last course of treatment and not on previous occasions.Much still remains to be learned concerning possible antigenic variants of factor VIII and the immunological mechanisms of inhibitor development.

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Inhibitor of Factor VIII in Mild Haemophilia

Cited by 30 publications

References 10 publications

Factor VIII antibody in a patient with mild haemophilia

Factor VIII antibody in a patient with mild haemophilia

Coagulation Factor Variants

Inhibitor of Factor Viii in Mild Haemophilia

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