Humate-P for treatment of von Willebrand disease [letter]

Czapek, EE; Gadarowski, JJ Jr; Ontiveros, JD; Pedraza, JL

doi:10.1182/blood.v72.3.1100.1100

Cited by 11 publications

(10 citation statements)

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“…Most hemophilia specialists now prefer such concentrates, or cryoprecipitate obtained from a small number of donors, to conventional multidonor cryoprecipitate therapy [2 1 1. One European factor VIII concentrate, which is expensive and currently unavailable in this country, has been reported to contain active vWF [22]. Unfortunately, most concentrates fail 300 - to correct the hemostatic abnormality in von Willebrand's disease [23] and none contain therapeutic quantities of fibrinogen [24] so that cryoprecipitate remains the usual treatment for these deficiencies.…”

Section: Discussionmentioning

confidence: 99%

Treatment of von willebrand's disease and hypofibrinogenemia with single donor cryoprecipitate from plasma exchange donation

1989

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Conventional replacement therapy for hypofibrinogenemia and von Willebrand's disease requires multiple donor exposures and a correspondingly high risk of blood-borne infection. We describe the collection and successful use of cryoprecipitate derived from a single donor by plasma exchange donation to support such patients through major hemostatic stresses. The father of an epileptic patient with von Willebrand's disease produced cryoprecipitate containing 23,546 units of von Willebrand factor (vWF) in nine desmopressin-stimulated donations; this provided total factor replacement for neurosurgery to remove a seizure focus. The average yield was 2,616 units per donation and the average VWF concentration in cryoprecipitate was 17.7 units/ml. The husband of a hypofibrinogenemic patient with a history of postpartum hemorrhage provided cryoprecipitate containing 13.4 g of fibrinogen in five donations; this supported his wife through parturition without recourse to other blood products. The average yield was 2.7 g per donation, and the average fibrinogen concentration was 15.3 g/liter. Plasma exchange donation is a practical alternative source for cryoprecipitate. It can provide vWF and fibrinogen that carry a reduced risk of infectious disease transmission.

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Section: Discussionmentioning

confidence: 99%

Treatment of von willebrand's disease and hypofibrinogenemia with single donor cryoprecipitate from plasma exchange donation

1989

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“…Haemate P is characterized by its high content of VWF, with a ratio of approximately 2.4 IU VWF:ristocetin cofactor activity (VWF:RCo) for each IU of FVIII:C. So although Haemate P was first developed for use in haemophilia A patients, reports of its use in VWD began appearing by the mid to late 1980s [20,[35][36][37][38][39][40][41]. Prior to the availability of Haemate P, it was generally believed that FVIII concentrates were not effective in VWD because of the absence of high molecular weight (HMW) VWF multimers in the concentrate preparations.…”

Section: Novel Therapy For Vwdmentioning

confidence: 99%

Haemate^®P von Willebrand factor/factor VIII concentrate: 25 years of clinical experience

Schramm¹

2008

Haemophilia

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Although von Willebrand disease (VWD) has a very long history, our understanding and treatment of the bleeding disorder has only evolved during the past 50 years or so. It was not until the 1920s that VWD was first recognized as a disease separate from that of classical haemophilia. It then took another 30 years before the first effective treatment was developed. Since then, the medical management of VWD has evolved considerably, but not without its ups and downs. One of the key milestones in the evolution of the treatment of VWD was the development of Haemate P/Humate-P (CSL Behring) - the first virus-inactivated factor VIII plasma product. For 25 years, this concentrate has demonstrated excellent clinical efficacy and safety for patients with VWD and for those with haemophilia. This article provides an historical overview of the early landmark efforts to ensure a safe plasma-derived replacement product and outlines the clinical evolution in the use of Haemate P.

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“…Nineteen patients with type I or I1 vWD have been treated with Hemate P@ (a pasteurized intermediate-purity concentrate manufactured by Behringwerke, Marburg, FRG) which normalized the BT measured with the relatively insensitive Duke method [27,31,32]. In 2 additional cases with type I and type IIA vWD, the more sensitive Ivy BT was also normalized [33]. Hemate P lacks a small proportion of the high-molecular-weight vWF multimers present in normal plasma ( fig.…”

Section: Biological Effects Of Concentratesmentioning

confidence: 99%

“…Virus-Inactivated Plasma Concentrates in von Willebrand Disease shortened but not normalized by the concentrate. Employing intermediate-purity concentrates, 36 patients with various types and subtypes of vWD have been treated successfully for spontaneous soft-tissue and mucosal hemorrhages (2 hemarthroses, 2 muscle hematomas, 2 menorrhagias, 8 epistaxes, 4 gastrointestinal hemorrhages) or for the prevention of bleeding during major surgery (n =lo), minor surgery (n = 4) or tooth extraction (n = 4) [31][32][33][34][35]37,38]. Hence, the available clinical data appear to indicate that virus-inactivated FVIII-vWF concentrates can be successfully used for the treatment of vWD, particularly for soft-tissue and postoperative bleeding.…”

Section: Rodeghiero/castaman/meyer/mannuccimentioning

confidence: 99%

“…In these instances, virusinactivated concentrates should be preferred to cryoprecipitate. All the commercially available low-and intermediate-purity concentrates appear equally effective in correcting the FVIII defect, whereas Hemate P and 8Y [33][34][35]37,38]. BT correction denotes reaching normal values; partial correction, shortening of 50% or more in comparison to basal values; no effect, no shortening or shortening of less than 50% in comparison to preinfusion values.…”

Section: Therapeutic Guidelinesmentioning

confidence: 99%

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

et al. 1992

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In von Willebrand disease, the goal of treatment is to correct the two laboratory hallmarks of abnormal hemostasis, i.e. the deficiency of factor VIII (FVIII) and the prolonged bleeding time (BT). Since desmopressin (DDAVP) is able to achieve both these goals in the majority of patients, it is the treatment of choice. Some patients, however, are not responsive or become refractory to DDAVP. In these, blood products maintain an important therapeutic role, and there is a need to assess the efficacy of the recently available virus-inactivated plasma concentrates, which contain both FVIII and von Willebrand factor and carry a low risk of transmitting blood-borne viruses. Our survey of the data reported in the literature indicates that all available concentrates are similarly effective in attaining high and sustained levels of FVIII after infusion. Although they often shorten or normalize the prolonged BT, that effect is less uniform. Since concentrates appear efficacious in the majority of clinical situations that require the use of blood products, they should be preferred, because of their greater safety, to cryoprecipitate produced by blood banks, which cannot be virus inactivated.

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Humate-P for treatment of von Willebrand disease [letter]

Cited by 11 publications

References 0 publications

Treatment of von willebrand's disease and hypofibrinogenemia with single donor cryoprecipitate from plasma exchange donation

Treatment of von willebrand's disease and hypofibrinogenemia with single donor cryoprecipitate from plasma exchange donation

Haemate^®P von Willebrand factor/factor VIII concentrate: 25 years of clinical experience

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

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Humate-P for treatment of von Willebrand disease [letter]

Cited by 11 publications

References 0 publications

Treatment of von willebrand's disease and hypofibrinogenemia with single donor cryoprecipitate from plasma exchange donation

Treatment of von willebrand's disease and hypofibrinogenemia with single donor cryoprecipitate from plasma exchange donation

Haemate®P von Willebrand factor/factor VIII concentrate: 25 years of clinical experience

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

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Haemate^®P von Willebrand factor/factor VIII concentrate: 25 years of clinical experience