Replacement Therapy with Virus- Inactivated Plasma Concentrates in von Willebrand Disease

Rodeghiero, Francesco; Castaman, Giancarlo; Meyer, Dominique; Mannucci, Pier Manuccio

doi:10.1159/000462201

Cited by 24 publications

(35 citation statements)

References 6 publications

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“…Intermediate purity FVIII-vWF concentrates have been increasingly used either on demand or to prevent a bleeding event, proving especially useful to treat soft tissue and post-operative bleeding [9]. The main products used at RFHHC over the last 17 years have been Haemate P (Centeon) and 8Y (BPL).…”

Section: Discussionmentioning

confidence: 99%

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Audit of clinical management of von Willebrand disease during 1997 at a single institution and review of treatment patterns between 1980 and 1997

Nitu-Whalley¹,

Miners²,

Lee³

1999

Haemophilia

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In 1997 the UK Haemophilia Centre Directors Organization published the guidelines for diagnosis and management of von Willebrand disease (vWD). The guidelines stated that desmopressin (DDAVP) should be used in type 1 and type 2N vWD, that it might be useful in other types 2 vWD and that it is ineffective in type 3 vWD. If patients are unresponsive to DDAVP or if it is contraindicated, the treatment of choice is clotting factor concentrates (CFC). In the light of these guidelines, we audited our practice for 1997. Furthermore, we undertook a retrospective review of the changing patterns of treatment of vWD between 1980 and 1997. During 1997, 10 patients with vWD received DDAVP and another 30 patients were treated with CFC (a total of 1.2 million IU): Haemate P (Centeon, Germany) and/or 8Y (BPL, Elstree, UK). Few patients had clear contraindications to DDAVP, but several patients with type 1 and 2 vWD received CFC on the basis of age or reduced levels of von Willebrand factor - where DDAVP was considered suboptimal for adequate haemostasis. However, this assumption was made without a preliminary test dose to assess the response to DDAVP. The analysis of treatment of vWD for the past 17 years showed that cryoprecipitate was discontinued from use in the early 1990s and that both DDAVP and CFC usage has been on the increase. In conclusion, the audit illustrated a general good adherence to guidelines but it highlighted the need for a DDAVP test before using CFC.

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Section: Discussionmentioning

confidence: 99%

“…However, they have less consistent results in correcting bleeding time and are therefore less effective in controlling mucosal bleedings [9]. All plasma concentrates correct FVIII levels and as a result they are usually ef®cacious in soft tissue and postoperative bleeding.…”

mentioning

confidence: 99%

Audit of clinical management of von Willebrand disease during 1997 at a single institution and review of treatment patterns between 1980 and 1997

Nitu-Whalley¹,

Miners²,

Lee³

1999

Haemophilia

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“…The effect of FVIII concentrates on the vWF:RCof and the template BT is less consistent, and improvements in the BT are usually partial and transient (95). Nonetheless, Humate P ® (Centeon), Koate HP ® (Bayer), and Alphanate ® (Alpha Therapeutics), three plasma-derived products that are commercially available at this time in the United States, improve each of these laboratory parameters in the majority of type 3 vWD patients (94, 96, 96a).…”

Section: Therapeutic Modalitiesmentioning

confidence: 99%

Von Willebrand's Disease

Ewenstein

1997

Annu. Rev. Med.

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von Willebrand's disease (vWD) arises from abnormalities in von Willebrand factor (vWF), an adhesive glycoprotein uniquely involved in key aspects of both primary and secondary hemostasis. The current classification distinguishes disorders arising from partial (type 1) or complete (type 3) deficiencies and from qualitative defects (type 2). Type 2 vWD is further divided into four subtypes (A, B, N, and M), reflecting distinct classes of functional abnormalities. Mis-sense mutations account for most of type 2 vWD, whereas major disruptions in the vWF gene produce type 3 variants. The molecular basis of type 1 vWD is largely undefined. The laboratory diagnosis of vWD and its several variants is made on the basis of immunologic and functional studies of vWF, factor VIII levels, and specialized electrophoretic analysis (multimer gels). The mainstay of therapy for most patients with vWD in desmopressin, a pharmacologic agent that stimulates the release of endogenous pools of vWF. Cryoprecipitate and selected factor VIII concentrates are useful sources of exogenous vWF for the treatment of patients unresponsive to this desmopressin.

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“…The cost of HIV is high in both developed and developing countries [12][13][14]. Concentrates have been shown to be safe against hepatitis [15] and effective in attaining normal levels of fVIII [16,17]. Concentrates have been shown to be safe against hepatitis [15] and effective in attaining normal levels of fVIII [16,17].…”

Section: Introductionmentioning

confidence: 99%

An economic model of haemophilia in Mexico

et al. 2004

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A model was developed to assess the lifetime costs and outcomes associated with haemophilia in Mexico. A retrospective chart review of 182 type A haemophiliacs was conducted for patients aged 0-34 years receiving one of three treatments: (i) cryoprecipitate at clinic; (ii) concentrate at home; or (iii) concentrate at clinic. Patients treated at home experienced 30% less joint damage, used 13-54% less factor VIII, had four times fewer clinic visits, and utilized half as many hospital days than those treated at a clinic. For cryoprecipitate at clinic patients, the annual incidence rates of HCV and HIV were calculated to be 3.6% and 1.4% respectively. The life expectancy for patients receiving cryoprecipitate and those receiving concentrate was estimated to be 49 years and 69 years respectively, with 58% of cryoprecipitate patients predicted to die of AIDS before age 69. Across the lifespan, the average annual cost of care was US$11,677 (MN$110,464) for cryoprecipitate at clinic patients, US$10,104 (M$95,580) for concentrate at home patients and US$18,819 (MN$178,027) for concentrate at clinic patients. Using a 5% discount rate, the incremental lifetime cost per year of life added for treatment with concentrate at home compared with cryoprecipitate at a clinic was US$738 (MN$6981). Rank order stability analysis demonstrated that the model was most sensitive to the cost of fVIII. These results indicate that treatment with concentrate at home compared with cryoprecipitate at a clinic substantially improves clinical outcomes at reduced annual cost levels.

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Replacement Therapy with Virus- Inactivated Plasma Concentrates in von Willebrand Disease

Cited by 24 publications

References 6 publications

Audit of clinical management of von Willebrand disease during 1997 at a single institution and review of treatment patterns between 1980 and 1997

Audit of clinical management of von Willebrand disease during 1997 at a single institution and review of treatment patterns between 1980 and 1997

Von Willebrand's Disease

An economic model of haemophilia in Mexico

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