Efficacy of porcine factor VIII in the management of haemophiliacs with inhibitors

Ciavarella, N.; Antoncecchi, S.; Ranieri, P

doi:10.1111/j.1365-2141.1984.tb06111.x

Cited by 33 publications

(23 citation statements)

References 8 publications

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“…This is one of the largest studies of factor VIII inhib itors carried out to date [7][8][9]. All 4 studies have similar ranges.…”

Section: Resultsmentioning

confidence: 99%

“…The purifica tion procedure of porcine factor VIII has been improved since 1980 by using ion exchange chromatography with polyelectrolytes [6] (Hyate:C®, Porton Products, UK). This new concentrate has been extensively used in the UK [7], Italy [8,9], the USA [10] and elsewhere [11,12], It has even been used for home therapy and the induction of immune tolerance in a small number of patients [13].…”

Section: Introductionmentioning

confidence: 99%

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Comparison of Anti-Human and Anti-Porcine Factor VIII Inhibitor Levels in 63 Patients with Severe Haemophilia A

Fiks-Sigaud¹,

Bendelac²,

Parquet³

et al. 1993

Vox Sanguinis

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The levels of anti-human and anti-porcine factor VIII inhibitors, measured in 63 severe haemophilia A patients, lay in the ranges of <0.2-2,600 and <0.2-1,300 Bethesda units per ml (BU/ml), respectively, with a median cross-reactivity of 33%. In 4 patients, human and porcine inhibitor levels were determined using both plasma, either human or porcine, and factor VIII concentrate, either very high purity human or porcine (Hyate:C®). A good correlation between titres was found, whatever the source of factor VIII (plasma or concentrate). The cross-reactivity varies from 0 to over 100%, indicating that the evaluation of both human and porcine inhibitors should be mandatory before any treatment with Hyate:C. Results show that of the 46 patients with human inhibitor of more than 5 BU/ml, 21 (46%) with a low porcine inhibitor (<5 BU/ml) could benefit from Hyate:C.

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“…This is one of the largest studies of factor VIII inhib itors carried out to date [7][8][9]. All 4 studies have similar ranges.…”

Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Comparison of Anti-Human and Anti-Porcine Factor VIII Inhibitor Levels in 63 Patients with Severe Haemophilia A

Fiks-Sigaud¹,

Bendelac²,

Parquet³

et al. 1993

Vox Sanguinis

View full text Add to dashboard Cite

show abstract

“…The highest quality evidence relating to the use of highly purified pFVIII to treat acute bleeding episodes comes from two uncontrolled prospective studies [19,28].…”

Section: Highly Purified Pfviii To Control Acute Bleedingmentioning

confidence: 99%

“…Ciavarella et al [28] studied the use of highly purified pFVIII in five haemophiliacs with inhibitors: four were high responders, and none had previously been treated with ‘first generation’ pFVIII concentrates (see Table 5 for details). They received pFVIII at a quantity corresponding to the calculated porcine neutralizing units plus the units required for haemostasis (40 U kg −1 12 h −1 for mucous membrane bleeding, 30 U kg −1 12 h −1 for haemarthrosis and haematoma).…”

Section: Highly Purified Pfviii To Control Acute Bleedingmentioning

confidence: 99%

Control of bleeding in patients with haemophilia A with inhibitors: a systematic review

et al. 2003

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This paper reports a systematic review of the best available evidence of clinical effectiveness in the treatment of acute bleeding in haemophilia A patients with inhibitors. Because of the lack of randomized controlled trials (RCTs) on this topic, broad inclusion criteria with regard to study design were applied in order to assess the best available evidence for each intervention. Because of the clinical and methodological heterogeneity of the evidence, it was not appropriate to pool data across studies; instead, data were synthesized using tabulation and qualitative narrative assessment. No evidence was found to support the use of high-dose factor VIII (FVIII) in bleeding episodes. However in surgery it was found to be highly successful (100%) for low-titre, low-responding inhibitors although not reliable for high-responding inhibitors. Porcine FVIII (pFVIII) was effective in the control of severe bleeding episodes with high-titre or high-responding inhibitors (100%) and in 60-90% of surgical procedures. Activated prothrombin complex concentrates (APCCs) appear to be more effective than prothrombin complex concentrates (PCCs) in the control of mild to severe bleeding episodes. There was no good evidence for the use of PCCs in surgery. APCCs controlled bleeding in approximately 90% of surgical episodes. Recombinant factor VIIa (rFVIIa) controlled 70-100% of mild to severe bleeding episodes with high-responding inhibitors, and achieved better results when used early. It was effective in 60-100% of surgical episodes. Doses varied from study to study, and side-effects from mild to infrequent but serious adverse events were reported. The quality of the evidence is variable. Limited evidence relating to other treatment options is also included in the review.

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“…These alloantibodies develop in response to the human FVIII, both plasma-derived and recombinant, which is used to treat these patients. The success of this in treating both congenital [10][11][12][13] and acquired haemophilia [14][15][16][17] is related largely to the fact that antibodies directed against human FVIII show lower crossreactivity with porcine FVIII than with human FVIII [18][19][20]. Regardless of their precise aetiology, the neutralizing effect of these antibodies on infused human FVIII renders this treatment approach suboptimal to control bleeding in affected patients [9].…”

Section: Introductionmentioning

confidence: 99%

Optimization of storage conditions for diluted working solutions of porcine factor VIII and performance of the Bethesda assay for the determination of antiporcine FVIII inhibitor titres

et al. 2003

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The use of porcine factor VIII (FVIII) (Hyate:C, Ipsen) has proven to be very successful in treating patients with FVIII inhibitors. The best way to predict the usefulness of porcine FVIII therapy, and/or to estimate the appropriate treatment dose in a given patient, is to measure the patient inhibitor titre against porcine FVIII with the Bethesda assay, using porcine FVIII as the source of FVIII in the assay. The goals of the present study were to (1) find the optimal storage temperature, diluent and concentration for a working solution of porcine FVIII to be used as the source of FVIII for the porcine Bethesda assay, (2) assess the reliability of the labelled FVIII units in the preparation of such working solutions of porcine FVIII and (3) compare the inhibitor titres determined by the Bethesda assay using both porcine and human standard reference curves for measuring residual FVIII. The results of the present study demonstrate that a ready-to-use working solution of 1 U mL(-1) of Hyate:C diluted in human FVIII deficient plasma, either containing or deficient in von Willebrand factor antigen, is stable for up to 12 months, at -20 degrees C. The preparation of the 1 U mL(-1) working solution could be reliably calculated based on the units indicated on the vial label. Finally, using the human standard curve yields similar results to using the porcine standard curve for measuring any titre of allo- or auto-antibody against FVIII in the Bethesda assay, using Hyate:C as the source of FVIII. These findings are of practical value when performing a porcine FVIII-based Bethesda assay.

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Efficacy of porcine factor VIII in the management of haemophiliacs with inhibitors

Cited by 33 publications

References 8 publications

Comparison of Anti-Human and Anti-Porcine Factor VIII Inhibitor Levels in 63 Patients with Severe Haemophilia A

Comparison of Anti-Human and Anti-Porcine Factor VIII Inhibitor Levels in 63 Patients with Severe Haemophilia A

Control of bleeding in patients with haemophilia A with inhibitors: a systematic review

Optimization of storage conditions for diluted working solutions of porcine factor VIII and performance of the Bethesda assay for the determination of antiporcine FVIII inhibitor titres

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