Failure of AHF concentrate to control bleeding in von Willebrand's disease

Potter, Elizabeth V.

doi:10.1016/0002-9343(76)90751-8

Cited by 86 publications

(30 citation statements)

References 6 publications

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“…On the other hand, the effect of concen trates on the prolonged bleeding time -the laboratory hallmark of the primary hemosta sis defect in von Willebrand disease -is usual ly not consistent and limited to the early post infusion period [1,3]. As a result, some types of hemorrhages (particularly in the mucosal tracts) are sometimes not properly controlled [4,5].…”

Section: Introductionmentioning

confidence: 99%

Biochemical Characteristics of Therapeutic Plasma Concentrates Used in the Treatment of von Willebrand Disease

Mannucci

1994

Pathophysiol Haemos Thromb

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Therapeutic plasma concentrates containing von Willebrand factor (vWF) lack the largest, most hemostatically active multimers. In order to evaluate whether this abnormality results from proteolysis during manufacturing, we have analyzed the subunit structure of vWF in 21 commercial products and found a marked reduction in the relative content of intact 225 kD subunit, paralleled by an increase in the proteolytic fragments normally present in plasma, particularly that of 176 kD. There was no heightened vWF fragmentation in blood-bank cryoprecipitate prepared from platelet-poor, single-donor plasma; in contrast, there was a marked degree of fragmentation in cryoprecipitate prepared from pooled plasmapheresis plasma representing the starting fraction for the production of commercial concentrates. In cryoprecipitate prepared experimentally from plasma containing varying numbers of platelets, the degradation of vWF was proportional to the platelet count but was greatly diminished by adding protease inhibitors to plasma. On the basis of these findings, we postulate that the loss of the largest vWF multimers in commercial concentrates results from the use of poorly centrifuged plasmapheresis plasma containing an excessive number of residual platelets and leukocytes.

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Section: Introductionmentioning

confidence: 99%

Biochemical Characteristics of Therapeutic Plasma Concentrates Used in the Treatment of von Willebrand Disease

Mannucci

1994

Pathophysiol Haemos Thromb

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“…However, alarmingly high proportions of haemophiliacs with inhibitors have been re ported, possibly in relation with more in tensive treatment [2]. Furthermore, minor qualitative differences of factor VIII con centrates have become clinically important, since they are not all equally suitable for treatment of von Willebrand disease and re lated disorders [6].…”

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confidence: 99%

Variable Degradation of Factor VIII-Related Protein in Lyophilised Concentrates of Antihaemophilic Factor (AHF)

Jakab¹,

Pflugshaupt²,

Furlan³

et al. 1978

Vox Sanguinis

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Factor VIII-related properties (coagulant = Vili:C, ‘Willebrand’ factor = VIII R: WF, antigen = Vili R: AG) are measured in a constant proportion in normal plasma and certain preparations of highly purified factor VIII (relative ratios: 0.5-1.5). We tested these activities in some commercial, lyophilised concentrates of factor VIII and found a variable increase of the ratio Vili R: AG/VIII R: WF. The relative increase of VIII R:AG, and/or loss of Vili R: WF, was attributed to variable degradation of factor VIII-related protein(s) which was directly visualised by electrophoresis on 2.75% polyacrylamide gels in the presence of sodium dodecyl sulphate.

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“…The hemostatic efficacy of vWf is directly pro portional to its multimer size. Previous stud ies have shown that commercial factor VIII/vWf concentrates lack the high-molecu lar-weight multimers [6,26] and they fail to correct the hemostatic defects in vWD [27,28] and in platelet-type vWD [9], It is appar ent that cryoprecipitate is hemostatically ef fective in these disorders. However, recent studies have revealed that transmission of the AIDS agent, human immunodeficiency virus, occurs through exposure to both nonheated factor VIII/vWf concentrates and cryoprecipitate [ 11,12], The present study was undertaken to seek safer replacement therapy in vWD including platelet-type vWD.…”

Section: Discussionmentioning

confidence: 99%

Heat-Treated Factor VIII/von Willebrand Factor Concentrate in Platelet-Type von Willebrand’s Disease

Takahashi

Tatewaki²,

Nagayama³

et al. 1987

Pathophysiol Haemos Thromb

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Cryoprecipitate has proved to correct the hemostatic defects in von Willebrand’s disease (vWD) and platelet-type vWD. However, recent studies have revealed that transmission of the AIDS retrovirus (HIV) occurs through exposure to blood products including cryoprecipitate. Treatment with heat-treated factor VlII/von Willebrand factor (vWf) concentrates may have certain advantages over treatment with nonheated products, if these preparations are efficacious in these disorders. We found that a commercially available factor VIII/vWf concentrate, Haemate P, contained the high-molecular-weight multimers of vWf and had a ratio of ristocetin cofactor (RCof) to vWf antigen (vWf: Ag) close to unity. In addition, its capacity to directly induce aggregation of platelet-type vWD platelets in vitro was similar to that for cryoprecipitate. When infused into a patient with platelet-type vWD, Haemate P shortened the prolonged bleeding time and caused spontaneous platelet aggregation in vitro with a mild diminution of platelet count. These results indicate that some of the heat-treated factor VIII/vWf concentrates may provide a safer, yet still effective, treatment for platelet-type vWD.

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Failure of AHF concentrate to control bleeding in von Willebrand's disease

Cited by 86 publications

References 6 publications

Biochemical Characteristics of Therapeutic Plasma Concentrates Used in the Treatment of von Willebrand Disease

Biochemical Characteristics of Therapeutic Plasma Concentrates Used in the Treatment of von Willebrand Disease

Variable Degradation of Factor VIII-Related Protein in Lyophilised Concentrates of Antihaemophilic Factor (AHF)

Heat-Treated Factor VIII/von Willebrand Factor Concentrate in Platelet-Type von Willebrand’s Disease

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