scholarly journals The Prevalence of von Willebrand Disease and Significance of in Vitro Bleeding Time (PFA-100) in von Willebrand Disease Screening in the İzmir Region

2013 ◽  
Vol 30 (1) ◽  
pp. 40-47 ◽  
Author(s):  
Fatih Şap ◽  
Tülay Kavaklı ◽  
Kaan Kavaklı ◽  
Ceyhun Dizdarer
Keyword(s):  
Bleeding Time ◽  
Disease Screening ◽  
Von Willebrand Disease ◽  
Von Willebrand

Patients with isolated prolonged in vitro bleeding time

2011 ◽  
Vol 31 (S 01) ◽  
pp. S64-S68
Author(s):  
M. Stein ◽  
K. Scholz ◽  
B. Llugaliu ◽  
G. Asmelash ◽  
W. Miesbach ◽  
...  
Keyword(s):  
Platelet Function ◽  
Von Willebrand Factor ◽  
Bleeding Time ◽  
Von Willebrand Disease ◽  
Treatment Concept ◽  
Main Symptom ◽  
Platelet Function Disorders ◽  
Von Willebrand ◽  
Willebrand Factor

SummaryIn patients with isolated prolonged in vitro bleeding time there is no standardised treatment concept. With this study we characterized the extent of bleeding symptoms. Patients, methods All diagnoses known to cause prolonged in vitro bleeding time (PFA-100) (epinephrine-cartridge >160 s, ADP-cartridge > 120 s) have been excluded, such as platelet function disorders, effects of medications, nutrition or von Willebrand disease. 75 patients (77%, n = 58 women; 23%, n = 17 men, median age 46 (16–81) years were included. All bleeding symptoms have been stored in a databank with help of a comprehensive questionnaire. Results 78% (n = 54) of all patients reported of having had an operation, 69.8% (n = 37) of them described postoperative bleedings (p = 0.0373). 13.5% (n = 5) of the 54 could remember having been randomly treated by the administration of a transfusion and only 2.7% (n = 1) were treated by substitution of von Willebrand factor. 71% (n = 51) patients indicated haematoma (p = 0.8116). About 33.8% (n = 24) patients had gum bleeding and 40.8% (n = 29, p = 0.7808) patients reported bleeding after the dentist. 41.4% (n = 29) patients suffered under frequent epistaxis (p = 0.0212). There was no correlation between prolonged epinephrine bleeding time to VWF : Ag (rho = 0.16) nor to VWF : RCo (rho = 0.12) nor between prolonged epinephrine and ADP bleeding time (rho = 0.01) nor to ROTEM® analysis. Conclusion Patients with isolated prolonged PFA are mainly women and can be affected by all kinds of bleedings while haematoma is the main symptom. VWD might not be causal

A randomised pilot trial of the anti-von Willebrand factor aptamer ARC1779 in patients with type 2b von Willebrand disease

2010 ◽  
Vol 104 (09) ◽  
pp. 563-570 ◽  
Author(s):  
Petra Paulinska ◽  
Petra Jilma-Stohlawetz ◽  
James Gilbert ◽  
Renta Hutabarat ◽  
Paul Knöbl ◽  
...  
Keyword(s):  
Pilot Trial ◽  
Coagulation Factor ◽  
Von Willebrand Disease ◽  
Double Blind ◽  
Clinical Trial Registration ◽  
Maximal Increase ◽  
Double Blind Design ◽  
Von Willebrand

SummaryDesmopressin aggravates thrombocytopenia in type 2B von Willebrand disease (VWF type 2B) by release of large and hyper-adhesive von Wille-brand Factor (VWF) multimers. This pilot study investigated whether the anti-VWF aptamer ARC1779 can prevent desmopressin-induced thrombocytopenia and interferes with the excessive VWF turnover in patients with VWF type 2B. Concentration effect curves of ARC1779 were established for five patients in vitro and two patients with VWF type 2B were treated by infusion of ARC1779, desmopressin, or their combination in a randomised, controlled, double-blind design. ARC1779 concentrations in the range of 1–3 μg/ml blocked free A1 domain binding sites by 90% in vitro. In vivo, desmopressin alone induced a profound (-90%) drop in platelet counts in one of the patients. ARC1779 (4–5 μg/ml) completely inhibited VWF A1 domains and prevented this desmopress-in-induced platelet drop. Desmopressin alone increased VWF antigen two- to three-fold, accompanied by concordant changes in VWF Ristocetin cofactor activity (RCo) and coagulation factor VIII activity. ARC1779 substantially enhanced the desmopressin-induced maximal increase in these parameters, and improved multimer patterns. No treatment related adverse events were observed and no bleeding occurred despite marked thrombocytopenia. These data provide first proof of concept in humans and evidence that ARC1779 is a potent inhibitor of VWF. ARC1779 prevented the rapid consumption of VWF multimers together with agglutinated platelets that occurred in response to desmopressin challenge in patients with VWD type 2B.Clinical Trial registration number: NCT00632242.

Clinical cases of using coagulation factor VIII with von Willebrand factor in parturient women with type III von Willebrand disease

2020 ◽  
Author(s):  
И.В. Куртов ◽  
Е.С. Фатенкова ◽  
Н.А. Юдина ◽  
А.М. Осадчук ◽  
И.Л. Давыдкин
Keyword(s):  
Factor Viii ◽  
Von Willebrand Factor ◽  
Coagulation Factor ◽  
Von Willebrand Disease ◽  
Coagulation Factor Viii ◽  
Vitro Fertilization ◽  
Type 3 ◽  
Von Willebrand ◽  
Willebrand Factor

Болезнь Виллебранда (БВ) может представлять определенные трудности у рожениц с данной патологией. Приведены 2 клинических примера использования у женщин с БВ фактора VIII свертывания крови с фактором Виллебранда, показана эффективность и безопасность их применения. У одной пациентки было также показано использование фактора свертывания крови VIII с фактором Виллебранда во время экстракорпорального оплодотворения. Von Willebrand disease presents a certain hemostatic problem among parturients. This article shows the effectiveness and safety of using coagulation factor VIII with von Willebrand factor for the prevention of bleeding in childbirth in 2 patients with type 3 von Willebrand disease. In one patient, the use of coagulation factor VIII with von Willebrand factor during in vitro fertilization was also shown.

Von Willebrand disease screening in women undergoing hysterectomy for heavy menstrual bleeding

Haemophilia ◽  
2019 ◽  
Vol 25 (3) ◽  
Author(s):  
Amanda E. Jacobson‐Kelly ◽  
Sara K. Vesely ◽  
Terah Koch ◽  
Janis Campbell ◽  
Sarah H. O’Brien
Keyword(s):  
Disease Screening ◽  
Von Willebrand Disease ◽  
Heavy Menstrual Bleeding ◽  
Menstrual Bleeding ◽  
Von Willebrand

FACTOR XIII IS NOT INVOLVED IN THE PLATELET ADHESION TO COLLAGEN

1987 ◽  
Author(s):  
K Kariya ◽  
Y Sawada ◽  
K Ueno ◽  
I Kudo ◽  
M Aihara ◽  
...  
Keyword(s):  
Factor Xiii ◽  
Platelet Adhesion ◽  
Bleeding Time ◽  
Type I Collagen ◽  
Rabbit Serum ◽  
Type I ◽  
Platelet Factor ◽  
Normal Plasma ◽  
Von Willebrand

We have reported that the adhesion of human formalin-fixed washed platelets (FWP) to collagen was enhanced by von Willebrand factor but inhibited by plasma fibronectin (Thromb. Res. 44, 1986). Recently, platelet factor XIII is reported to be a receptor for collagen (Saito, JBC, 261, 1986). To investigate the role of factor XIII in the interaction between platelets and collagen, effect of purified XIII or rabbit anti-XIIIa on the adhesion of FWP to collagen or on the in vitro bleeding time was studies. FWP adhesion was measured by either collagen-coated glass beads column or aggregometric method using bovine type I collagen (Ethicon Inc., Dr. Kronenthal). In vitro bleeding time was measured with Thrombostat-4000 (VDG-VONDERGOLTZ), in which citrated whole blood as a sample and ADP, CaCl2 and rat type I collagen as the reagents were used. Platelet adhesion to the collagen immobilized column (1,300 ug collagen, flow rate 5 ml/min) was not changed by the addition of purified XIII (Fibrogammin, Hoechst); the adhesion were 42.7 ± 1.7% in the presence of 1% human serum albumin, 42,0 ± 0.3%, 43,0 ± 1.4% in the presence of 1 or 2 u/ml factor XIII. Furthermore, the adhesion of FWP which was added by 1:100 rabbit anti-XIIIa was 42.3 ± 1.4% and not different from that of control rabbit serum (46.1 ± 1.3%). Similar results were also obtained with different technique using aggregometer. No significant change on in vitro bleeding time was observed after the addition of 1:100 rabbit anti-XIIIa to citrated normal blood. When the binding of factor XIII to the collagen was investigated by batch method, 17%, 23% and 54% of factor XHIa in normal plasma bound to 250, 500 and 1000 ug/ml collagen, respectively. These data suggest that factor XIII is not involved in the platelet adhesion to the type I fibrillar collagen, while factor XHIa in normal plasma binds to the collaeen.

scholarly journals Comparison of four virus-inactivated plasma concentrates for treatment of severe von Willebrand disease: a cross-over randomized trial

Blood ◽  
1992 ◽  
Vol 79 (12) ◽  
pp. 3130-3137 ◽  
Author(s):  
PM Mannucci ◽  
PM Tenconi ◽  
G Castaman ◽  
F Rodeghiero
Keyword(s):  
Plasma Levels ◽  
Bleeding Time ◽  
Von Willebrand Disease ◽  
Prolonged Bleeding Time ◽  
Randomized Design ◽  
Coagulant Activity ◽  
Von Willebrand ◽  
Willebrand Factor ◽  
Ristocetin Cofactor ◽  
Ristocetin Cofactor Activity

Abstract Until recently, cryoprecipitate has been the treatment of choice in patients with severe von Willebrand disease (vWD) because it can transiently correct low plasma levels of factor VIII coagulant activity (FVIII:C) and shorten or normalize the prolonged bleeding time (BT), the two laboratory hallmarks of the disease. However, cryoprecipitate may still transmit blood-borne viruses, whereas the development of virucidal methods have rendered plasma concentrates containing FVIII:C and von Willebrand factor (vWF) safer. To establish their potential usefulness in the treatment of vWD, we compared the effect of four virus-inactivated concentrates on FVII:C and vWF plasma levels and the BT (template method) in 10 patients with severe vWD using a crossover randomized design. The concentrates were an intermediate-purity, pasteurized FVIII-vWF concentrate; an intermediate-purity, dry-heated FVIII-vWF concentrate; a solvent/detergent-treated vWF concentrate, containing little FVIII; and a high-purity solvent/detergent-treated FVIII-vWF concentrate. All concentrates were equally effective in attaining normal and sustained levels of FVIII:C postinfusion, although peak levels were more delayed after the vWF concentrate. The effect of concentrates on the BT, however, was less uniform and satisfactory. The pasteurized FVIII-vWF concentrate transiently corrected, completely or partially, the BT in 8 of 10 patients, the dry-heated and solvent/detergent FVIII/vWF concentrates in five, whereas in no patient did the vWF concentrate correct the BT according to the criteria used in this study. These effects on the BT were not related to the plasma levels of ristocetin cofactor activity-attained postinfusion (100 U/dL or more in the majority of patients) or to the multimeric structure of vWF in concentrates (defective in larger multimers in all cases). In conclusion, even though virus-inactivated concentrates can be used to increase FVIII:C levels in patients with severe vWD, none of the concentrates studied by us consistently normalizes the BT in a sustained fashion.

scholarly journals New variant of von Willebrand disease type II with markedly increased levels of von Willebrand factor antigen and dominant mode of inheritance: von Willebrand disease type IIC Miami

Blood ◽  
1993 ◽  
Vol 82 (1) ◽  
pp. 169-175 ◽  
Author(s):  
MR Ledford ◽  
I Rabinowitz ◽  
JE Sadler ◽  
JW Kent ◽  
F Civantos
Keyword(s):  
Molecular Weight ◽  
Von Willebrand Factor ◽  
Bleeding Time ◽  
Autosomal Dominant ◽  
Disease Type ◽  
Von Willebrand Disease ◽  
Dominant Inheritance ◽  
New Variant ◽  
Von Willebrand ◽  
Willebrand Factor

A variant of von Willebrand disease (vWD) was identified in six members of a kindred spanning four generations. The proband was a 46-year-old woman with a lifelong history of bleeding, a prolonged bleeding time (> 15 minutes), markedly elevated von Willebrand factor (vWF) antigen (vWF:Ag = 2.09 U/mL), slightly reduced ristocetin cofactor activity, and a plasma vWF multimer pattern similar to that of vWD type IIC. Similar findings were observed in her three children, mother, and brother. In affected family members, platelet and plasma vWF multimer patterns were discrepant with higher molecular weight multimers observed in platelet vWF. Following a 1-Des-amino-8-D-arginine vasopressin (DDAVP) challenge, the proband failed to normalize her bleeding time even though vWF: Ag rose by 70% and higher molecular weight multimers were increased slightly. Genetic studies were consistent with autosomal dominant inheritance of a mutation within the vWF gene. By sequencing of cloned genomic DNA, mutations were excluded in exons 4, 5, 14, and 15, which encode regions of the vWF propeptide proposed to be important in multimer biosynthesis. Mutations also were excluded in exons 28 to 31, which encompass the known mutations that cause vWD types IIA, IIB, and B. This new variant of vWD, characterized by autosomal dominant inheritance, a qualitative defect that resembles vWD type IIC, and increased plasma vWF:Ag, was tentatively designated vWD type IIC Miami.

scholarly journals The RIIIS/J inbred mouse strain as a model for von Willebrand disease

Blood ◽  
1990 ◽  
Vol 76 (11) ◽  
pp. 2258-2265 ◽  
Author(s):  
JD Sweeney ◽  
EK Novak ◽  
M Reddington ◽  
KH Takeuchi ◽  
RT Swank
Keyword(s):  
Bleeding Time ◽  
Inbred Mouse ◽  
Inbred Mouse Strain ◽  
Von Willebrand Disease ◽  
Bleeding Tendency ◽  
Serotonin Content ◽  
Prolonged Bleeding ◽  
Prolonged Bleeding Time ◽  
Plasma Factor ◽  
Von Willebrand

Abstract Mice of the RIIIS/J inbred strain have prolonged bleeding times (greater than 15 minutes) after experimental injury when compared with normal C57BL/6J mice (1.8 minutes) and other strains of mice. The prolonged bleeding time was accompanied by normal platelet counts. Platelet aggregation with collagen and agglutination with ristocetin were not significantly altered in RIIIS/J mice. Also, platelets from RIIIS/J mice had normal serotonin content and normal numbers of dense granules by electron microscopy. Thus, the bleeding abnormality is not due to platelet storage pool deficiency as has been found in several other mouse mutants. The activated partial thromboplastin time (APTT) which plasma from RIIIS/J mice was prolonged compared with normal mice, and factor VIII:C activity and von Willebrand antigen levels were one half to one third that of normal mouse plasma. Factor XI activity was also significantly deficient (levels at 42% to 64% of normal). Plasma of RIIIS/J mice contained the full complement of multimers of von Willebrand factor, although each multimer was lower in concentration compared with that in normal mice. Platelet alpha-granule von Willebrand antigen levels were similar to those of normal mice. The prolonged bleeding time of RIIIS/J mice was corrected by treatment with desmopressin. Heterozygous C57BL/6J x RIIIS/J F1 animals had low plasma von Willebrand antigen levels like the RIIIS/J parent and had variable bleeding times. Inheritance of the bleeding tendency was as an incomplete dominant, autosomal trait. These data indicate the RIIIS/J strain is a suitable animal model for type IA von Willebrand disease.

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