Immunoadsorption for coagulation factor inhibitors: a retrospective critical appraisal of 10 consecutive cases from a single institution

Rivard, G. E.; Louis, James St.; Lacroix, Sébastien; Champagne, Martin A.; Rock, G.

doi:10.1046/j.1351-8216.2003.00814.x

Cited by 50 publications

(44 citation statements)

References 6 publications

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“…Underlying diseases for acquired hemophilia were found in six patients, two had an autoimmune disease (Cases 2 and 6), and four developed their inhibitor as paraneoplastic syndrome (Cases 1, 5, 7, and 8). The median number of IA treatments was 14 (range [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] performed during a treatment period of 6-35 days (Table I).…”

Section: Resultsmentioning

confidence: 99%

Immunoadsorption with single‐use columns for the management of bleeding in acquired hemophilia A: A series of nine cases

Brzoska¹,

Krause²,

Geiger³

et al. 2007

J of Clinical Apheresis

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The presented cases support our assumption that patients with acquired hemophilia A benefit from IA with disposable columns in a setting of acute bleeding. This modality of IA is able to eliminate inhibitors reliably and quickly. IA in general is substantially speeding up the progress of therapy preventing bleeding complications constantly threatening the patient and reducing the dosages of coagulation factor therapy. We encourage IA with disposable columns in all bleeding patients with acquired hemophilia to aggressively lower the inhibitors.

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Section: Resultsmentioning

confidence: 99%

Immunoadsorption with single‐use columns for the management of bleeding in acquired hemophilia A: A series of nine cases

Brzoska¹,

Krause²,

Geiger³

et al. 2007

J of Clinical Apheresis

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“…In contrast to hemophilia A, in which the cumulative incidence of inhibitor development ranges from 15%-30%, 15,16 the incidence of inhibitory Abs in patients with congenital FXIII deficiency is very rare and has been reported in only 5 patients treated with plasma-based products. 6,[17][18][19] The development of an inhibitor in hemophilia or congenital FXIII deficiency complicates continued management of the patient substantially; therefore, the present trial closely evaluated and monitored for Ab development to FXIII by testing patients monthly for anti-rFXIII Abs. No inhibitory Abs were found in this trial.…”

Section: Discussionmentioning

confidence: 99%

Recombinant factor XIII: a safe and novel treatment for congenital factor XIII deficiency

Inbal

Oldenburg²,

Carção

et al. 2012

Blood

110

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“…37 This modality may be useful in bleeding or presurgical patients with high titer inhibitors who have failed to respond to bypassing agents. Following pheresis or immunoadsorption, these patients should be treated with FVIII replacement to achieve hemostasis.…”

Section: Inhibitor Removalmentioning

confidence: 99%

Acquired Factor VIII Inhibitors: Pathophysiology and Treatment

Alice¹,

Carrizosa²

2006

Hematology

156

198

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Hemophilia A is classically caused by a congenital deficiency of factor VIII, but an acquired form due to inhibitors to factor VIII (FVIII) typically presents later in life. Patients who develop such acquired factor VIII inhibitors may present with catastrophic bleeding episodes, despite having no prior history of a bleeding disorder. Though the disorder is rare, it is known to cause significant morbidity and mortality. This review will focus on what is currently known about acquired hemophilia A, its pathogenesis, its associated etiologies, and its treatment. Factor VIII and Acquired InhibitorsFactor VIII functions as a cofactor to factor IXa in the tenase complex, and a deficiency of factor VIII thus reduces the generation of thrombin on the surface of activated platelets. Factor VIII is synthesized as a 330-kDa precursor protein with an A 1 -a 1 -A 2 -a 2 -B-a 3 -A 3 -C 1 -C 2 domain structure. 1After proteolytic processing, FVIII associates with von Willebrand Factor (vWF) in heterodimers of a heavy (A 1 -a 1 -A 2 -a 2 ) and a light (a 3 -A 3 -C 1 -C 2 ) chain associated by a metal ion interaction. Most acquired FVIII inhibitors bind to the A2, A3 or C2 domains.2,3 Anti-C2 antibodies disrupt the binding of FVIII to phospholipid and vWF, while antibodies to A2 and A3 interfere with FVIII binding to factor X and factor IXa.In congenital hemophilia A patients treated with factor VIII, alloantibodies to FVIII develop in 20-40% of patients. By contrast, acquired inhibitors/autoantibodies against FVIII in nonhemophiliacs occur in only about one case per million per year. 4 In acquired hemophilia, autoantibodies are characteristically non-complement fixing, nonprecipitating immunoglobulins from the IgG family that bind FVIII in a time-and temperature-dependent manner. 5While most alloantibodies inactivate FVIII in direct proportion to their concentration (first-order kinetics), acquired inhibitors/autoantibodies show a non-linear inactivation pattern (type II or second-order kinetics). The kinetics plots in Figure 1 show a linear inactivation of FVIII by a type 1 alloantibody, with eventual complete inhibition of the FVIII activity. By contrast, the type 2 antibody shows a rapid initial inactivation phase followed by a slower phase of equilibrium where some factor VIII can usually be measured.

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Immunoadsorption for coagulation factor inhibitors: a retrospective critical appraisal of 10 consecutive cases from a single institution

Cited by 50 publications

References 6 publications

Immunoadsorption with single‐use columns for the management of bleeding in acquired hemophilia A: A series of nine cases

Immunoadsorption with single‐use columns for the management of bleeding in acquired hemophilia A: A series of nine cases

Recombinant factor XIII: a safe and novel treatment for congenital factor XIII deficiency

Acquired Factor VIII Inhibitors: Pathophysiology and Treatment

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