Clinical use of Haemate® P in von Willebrand disease: A 25-year retrospective observational study

Miesbach, Wolfgang; Krekeler, S.; Wolf, Zsuzsanna; Seifried, Erhard

doi:10.1016/j.thromres.2014.12.017

Cited by 20 publications

(18 citation statements)

References 18 publications

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“…In the United States, VWD is typically treated with either Humate‐P49 or Wilate 50. Both products also contain plasma derived FVIII, although at different ratios (VWF:FVIII 2.4:1 for Humate‐P and 1:1 for Wilate).…”

Section: Treatment Of Vwdmentioning

confidence: 99%

Current issues in diagnosis and treatment of von Willebrand disease

Keesler

Flood

2018

Research and Practice in Thrombosis and Haemostasis

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Essentials Classification of VWD includes quantitative (types 1 and 3) and qualitative (type 2) variants.New assays of VWF‐platelet binding may improve accuracy in diagnosis.Collagen binding represents an additional function of VWF not measured by current tests.Treatment options for VWD include desmopressin, plasma‐derived, and recombinant VWF. Clinically, von Willebrand disease (VWD) presents as mucosal bleeding caused by a decreased quantity or quality of von Willebrand factor (VWF). Diagnosis of VWD requires careful consideration of patient specific factors, bleeding symptoms, and laboratory results. Patients with borderline low VWF levels remain challenging, given that low VWF is not necessarily a guarantee of bleeding, but is present in many patients with symptoms, and treatment of low VWF may improve bleeding. Laboratory diagnosis of VWD is complex and no single test can determine the presence or absence of functional VWF. Historically, VWF binding to platelet GPIbα was measured by the ristocetin cofactor assay (VWF:RCo); a new assay using platelet GPIbα in the absence of ristocetin (VWF:GPIbM) is gradually replacing the VWF:RCo due to improved accuracy in diagnosis. VWF binding to collagen is a separate function, and requires specific testing to determine if a collagen binding defect is present. Regardless of these laboratory complexities, clinicians can empirically treat VWD to alleviate bleeding symptoms by raising VWF levels through desmopressin or VWF concentrate. Recombinant VWF is now available, but clinicians may need to add an initial dose of FVIII when treating emergency bleeds.

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Section: Treatment Of Vwdmentioning

confidence: 99%

Current issues in diagnosis and treatment of von Willebrand disease

Keesler

Flood

2018

Research and Practice in Thrombosis and Haemostasis

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“…Recently, Miesbach and colleagues reported on a 25‐year retrospective observational study of Humate‐P in 71 VWD patients. They observed no allergies or transmission of viral infections and only one adverse reaction, a case of PE attributed to incorrect dosing (due to transcription error, the patient received three times the desired dose).…”

Section: Resultsmentioning

confidence: 99%

“…It was initially licensed in Germany in 1982 and subsequently approved in the United States in 1986 for the management of bleeding in patients with hemophilia A and later for VWD. Several publications have documented the efficacy of Humate‐P in VWD and hemophilia A …”

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confidence: 99%

Safety of a pasteurized plasma‐derived Factor VIII and von Willebrand factor concentrate: analysis of 33 years of pharmacovigilance data

et al. 2017

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BACKGROUND Haemate‐P/Humate‐P (Humate‐P) is a pasteurized human plasma‐derived concentrate containing both Factor VIII and von Willebrand factor for treatment of hemophilia A and von Willebrand disease (VWD). STUDY DESIGN AND METHODS We analyzed the safety of Humate‐P based on more than 33 years of postmarketing pharmacovigilance data, representing an estimated exposure of approximately 25,000 patient‐years. The analysis comprises reports of potential adverse drug reactions (ADRs) from all sources, reported as part of routine pharmacovigilance at CSL Behring. ADRs considered clinically relevant or potential risks of Humate‐P were identified based on defined and standardized Medical Dictionary for Regulatory Activities queries. Recognizing the limitations of spontaneous reporting, we also reviewed the literature, including clinical trials with mandatory reporting. RESULTS From 1982 to 2015, a total of 670 postmarketing cases had been reported via pharmacovigilance, for an overall reporting rate of approximately one ADR per 3900 administered standard doses. Of these cases, 343 involved ADRs considered clinically relevant risks (33 thromboembolic complications, 97 inhibitor formation, 110 hypersensitivity or allergic reactions) or potential risks (103 suspected virus transmissions) for Humate‐P. Most thromboembolic complications occurred in patients undergoing surgery or with other known risk factors. Inhibitor formation occurred mostly in patients with hemophilia A (24 cases were high titer). Most patients with hypersensitivity or allergic reactions had VWD. None of the reported suspected virus transmission cases were confirmed to be associated with Humate‐P. Reported results of company‐sponsored studies showed a low incidence of adverse events possibly or probably related to Humate‐P. CONCLUSIONS More than 33 years of pharmacovigilance data continue to support the safety of Humate‐P.

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“…Due to a transcription error, the patient received 65.22 IU FVIII/kg (6000 IU) Haemate ® P (instead of the recommended 21.74 IU FVIII/kg once‐daily dose) without consulting with the haemophilia centre, which led to a marked increase in VWF:Ag, VWF:RCo and FVIII. The patient developed bilateral pulmonary embolism on day 7 postsurgery .…”

Section: Special Instructions For the Management Of Surgerymentioning

confidence: 99%

Von Willebrand disease – the ‘Dos’ and ‘Don'ts’ in surgery

Miesbach

Berntorp

2016

European J of Haematology

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Von Willebrand disease (VWD) is the most common genetic bleeding disorder. VWD is caused by a deficiency or dysfunction of von Willebrand factor (VWF), a plasma protein that mediates the initial adhesion of platelets at sites of vascular injury and binds and stabilises coagulation factor VIII (FVIII) in the blood. Prophylaxis of surgical bleeding in patients with VWD requires consideration of the individual situation, including the type of procedure and the bleeding rate, before decisions about treatment type, dose, duration and adjunctive therapy with antifibrinolytics or antithrombotic prophylaxis can be made. Although desmopressin (DDAVP)-stimulated release of endogenous VWD is an effective treatment strategy in many patients, plasma concentrates containing VWF are the preferred option for most patients undergoing surgical procedures. Recommendations for the management of surgery in patients with VWD are summarised, including the severity of VWD and the type of the surgical procedure.

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Clinical use of Haemate® P in von Willebrand disease: A 25-year retrospective observational study

Cited by 20 publications

References 18 publications

Current issues in diagnosis and treatment of von Willebrand disease

Current issues in diagnosis and treatment of von Willebrand disease

Safety of a pasteurized plasma‐derived Factor VIII and von Willebrand factor concentrate: analysis of 33 years of pharmacovigilance data

Von Willebrand disease – the ‘Dos’ and ‘Don'ts’ in surgery

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