Bleeding Time and in vivo Platelet Adhesiveness in von Willebrand's Disease: Effect of Iontophoresis of Adenosine Diphosphate

Nature ◽  
1967 ◽  
Vol 215 (5107) ◽  
pp. 1302-1303 ◽  
Author(s):  
J. LESLIE
Keyword(s):  
Bleeding Time ◽  
Adenosine Diphosphate ◽  
Platelet Adhesiveness ◽  
Von Willebrand's Disease ◽  
Von Willebrand’S Disease ◽  
Disease Effect

The Spectrum of von Willebrand’s Disease

1967 ◽  
Vol 18 (01/02) ◽  
pp. 040-056 ◽  
Author(s):  
E. J Walter Bowie ◽  
P Didisheim ◽  
J. H Thompson ◽  
C. A Owen
Keyword(s):  
Factor Viii ◽  
Bleeding Time ◽  
Severe Bleeding ◽  
Platelet Adhesiveness ◽  
Platelet Activity ◽  
Von Willebrand's Disease ◽  
Von Willebrand’S Disease ◽  
The Third ◽  
Generation Test

SummaryPatients (from 5 kindreds) with variants of von Willebrand’s disease are described. In one kindred the depression of factor VIII was moderate (20 to 40% of normal) and transfusion of 500 ml of normal plasma led to an increase higher than anticipated and to an almost normal level of factor VIII 17 to 24 hrs later. This represents the usual type of von Willebrand’s disease.In the second kindred the concentration of factor VIII was less than 2 % of normal in the son and daughter, who had severe bleeding and hemarthroses.The third kindred was characterized by reduction of factor VIII and a long bleeding time as well as by a serum defect in the thromboplastin-generation test comparable to that seen in patients with hemophilia B, yet with normal levels of factors IX, X, and VII. The severity of the serum defect, the positive result with the Rumpel-Leede test, and the reduced platelet activity in the thromboplastin-generation test are all compatible with the diagnosis of thrombopathy or ‘‘thrombopathic hemophilia.” In two other kindreds, one patient had a long bleeding time and normal levels of factor VIII and another had a normal bleeding time and decrease of factor VIII. The last patient had the type of response to transfusion usually seen in von Willebrand’s disease.In four kindreds, platelet adhesiveness in vivo was found to be strikingly abnormal (virtually absent).It would appear, therefore, that von Willebrand’s disease forms a spectrum, and whether the kindreds reported simply reflect variations of a single genetic disease state or represent separate entities will be answered only by clarification of the underlying etiology of that disease.

Increased Activity of “Anti-Willebrand Factor” in Diabetic Plasma

1964 ◽  
Vol 11 (01) ◽  
pp. 027-037 ◽  
Author(s):  
A. E Ödegaard ◽  
B. A Skalhegg ◽  
A. J Hellem
Keyword(s):  
Platelet Adhesiveness ◽  
Von Willebrand's Disease ◽  
Von Willebrand’S Disease ◽  
Prolonged Bleeding Time ◽  
Platelet Reaction ◽  
Willebrand Factor ◽  
Increased Activity

SummaryIn a group of 25 patients with insulin-treated diabetes mellitus a marked increase in the ADP-induced platelet adhesiveness was demonstrated. This phenomenon was due to a plasmatic factor. In vitro-plasma from these patients restored the decreased ADP-induced platelet adhesiveness in von Willebrand’s disease.The factor was also effective in vivo, since transfusion of 450 ml diabetic plasma to a patient with von Willebrand’s disease normalized the decreased platelet adhesiveness and shortened the prolonged bleeding time. The role of this factor in the ADP platelet reaction as a cofactor together with calcium is stressed.

scholarly journals The heterogeneity of type IIA von Willebrand's disease: studies with protease inhibitors

Blood ◽  
1986 ◽  
Vol 68 (6) ◽  
pp. 1207-1212 ◽  
Author(s):  
J Batlle ◽  
MF Lopez Fernandez ◽  
M Campos ◽  
B Justica ◽  
C Berges ◽  
...  
Keyword(s):  
Bleeding Time ◽  
Relative Proportion ◽  
Clinical Expression ◽  
Von Willebrand's Disease ◽  
Von Willebrand’S Disease ◽  
Time Correction ◽  
Von Willebrand ◽  
Willebrand Factor

The absence of large von Willebrand factor (vWF) multimers from plasma is a characteristic of Type IIA von Willebrand's disease (vWD) and is thought to contribute to the clinical expression of this disorder. Recently, three IIA patients have been reported in whom intermediate and large multimers could be restored if blood were collected in 5 mm EDTA, 6 mmol/L N-ethylmaleimide, and 1 mmol/L leupeptin. This suggested that absence of large multimers resulted from in vitro proteolysis. We have now collected blood from ten Type IIA vWD patients in these inhibitors but were not able to detect large multimers in the plasma of any of them. In addition, intermediate-sized multimers were reduced or completely absent in all. The inclusion of inhibitors in the citrate anticoagulant, as compared to citrate alone, was found to increase the relative proportion of intermediate multimers in some patients but had no effect in others, and in none did it restore large multimers to plasma. The results with platelet vWF were more varied. Four patients showed an absence or decrease of large multimers, whereas in seven patients large multimers were present. When compared with citrate anticoagulant alone, the inclusion of inhibitors in the anticoagulant had little or no effect on the platelet multimeric pattern. 1-Deamino-8- D-Arginine Vasopressin (DDAVP) was administered to six patients from five families. Two patients from one family showed complete correction and a third patient showed almost complete correction of her bleeding time. Two patients showed minimal correction and one showed no detectable correction. An increase in multimer size after DDAVP tended to be associated with correction of the bleeding time. However, in no case did the largest multimers appear in plasma even in patients with complete bleeding time correction. The presence or absence of inhibitors in the anticoagulant had little or no effect on the multimeric pattern after DDAVP. These results indicate that Type IIA vWD is a heterogeneous disorder in which absence of largest and intermediate multimers is an in vivo phenomenon.

Immunologie Studies in von Willebrand’s Disease

1976 ◽  
Vol 35 (01) ◽  
pp. 110-119 ◽  
Author(s):  
Y Sultan ◽  
J Simeon ◽  
P Maisonneuve ◽  
J. P Caen
Keyword(s):  
Factor Viii ◽  
Bleeding Time ◽  
The Other ◽  
Von Willebrand's Disease ◽  
Factor Viii Related Antigen ◽  
Von Willebrand’S Disease ◽  
Short Period ◽  
Von Willebrand ◽  
Immunologic Properties

SummaryTwo patients with a severe von Willebrand’s disease characterized by no detectable factor VIII related antigen in their plasma received transfusions of cryoprecipitate. The bleeding time was corrected for a short period of time and returned to its pretransfusional value although the other parameters of the disease were still corrected. Electrophoretic and immunologic properties of factor VIII related antigen infused were determined serially after transfusion. Modifications of these properties occurred progressively after transfusion. The half disappearance time of F. VIIIR. A. was determined and found to be considerably shorter than in hemophilic recipients. This study suggests an alteration in vivo of F. VIIIR. A. infused into von Willebrand recipients.

scholarly journals The heterogeneity of type IIA von Willebrand's disease: studies with protease inhibitors

Blood ◽  
1986 ◽  
Vol 68 (6) ◽  
pp. 1207-1212 ◽  
Author(s):  
J Batlle ◽  
MF Lopez Fernandez ◽  
M Campos ◽  
B Justica ◽  
C Berges ◽  
...  
Keyword(s):  
Bleeding Time ◽  
Relative Proportion ◽  
Clinical Expression ◽  
Von Willebrand's Disease ◽  
Von Willebrand’S Disease ◽  
Time Correction ◽  
Von Willebrand ◽  
Willebrand Factor

Abstract The absence of large von Willebrand factor (vWF) multimers from plasma is a characteristic of Type IIA von Willebrand's disease (vWD) and is thought to contribute to the clinical expression of this disorder. Recently, three IIA patients have been reported in whom intermediate and large multimers could be restored if blood were collected in 5 mm EDTA, 6 mmol/L N-ethylmaleimide, and 1 mmol/L leupeptin. This suggested that absence of large multimers resulted from in vitro proteolysis. We have now collected blood from ten Type IIA vWD patients in these inhibitors but were not able to detect large multimers in the plasma of any of them. In addition, intermediate-sized multimers were reduced or completely absent in all. The inclusion of inhibitors in the citrate anticoagulant, as compared to citrate alone, was found to increase the relative proportion of intermediate multimers in some patients but had no effect in others, and in none did it restore large multimers to plasma. The results with platelet vWF were more varied. Four patients showed an absence or decrease of large multimers, whereas in seven patients large multimers were present. When compared with citrate anticoagulant alone, the inclusion of inhibitors in the anticoagulant had little or no effect on the platelet multimeric pattern. 1-Deamino-8- D-Arginine Vasopressin (DDAVP) was administered to six patients from five families. Two patients from one family showed complete correction and a third patient showed almost complete correction of her bleeding time. Two patients showed minimal correction and one showed no detectable correction. An increase in multimer size after DDAVP tended to be associated with correction of the bleeding time. However, in no case did the largest multimers appear in plasma even in patients with complete bleeding time correction. The presence or absence of inhibitors in the anticoagulant had little or no effect on the multimeric pattern after DDAVP. These results indicate that Type IIA vWD is a heterogeneous disorder in which absence of largest and intermediate multimers is an in vivo phenomenon.

Replacement Therapy in Von Willebrand’s Disease

1979 ◽  
Author(s):  
Y. Sultan
Keyword(s):  
Replacement Therapy ◽  
Surgical Procedures ◽  
Bleeding Time ◽  
Normal Values ◽  
Procoagulant Activity ◽  
Von Willebrand's Disease ◽  
Von Willebrand’S Disease ◽  
Bleeding Episodes ◽  
Severity Of The Disease

Replacement therapy in patients with VWD for bleeding episodes or surgical procedures is not as well defined as treatment of patients with Hemophilia. In severe forms of the disease although it is aesy to obtain high levels of FVIII procoagulant activity after transfusion with FVIII concentrate it is more difficult to achieve a normal bleeding time (B.T.) and moreover to keep it normal throughout surgical procedures and healing.The normalization of B.T. is dependent on the kind of FVIII concentrates used. To demonstrate this, several commercial preparations of FVIII have been tested for their content in FVIII R Ag and FVIII RWF in comparison with the procoagulant activity ; they have also been tested in vivo for their ability to correct B.T.. shortening of B.T. is also dependent on the quantities injected ; it is more efficient to infuse large doses once a day or every other day, than fractionated doses F It is also dependent on the severity of the disease and the number of transfusions the patient previously received. Some problems remain unsolved, specially when all the parameters of the disease reach normal values in the circulating blood although B.T. is still prolonged.

Effect of Plasma and Platelet Concentrate Infusions on Factor VIII, Platelet Adhesiveness, and Bleeding Time in Bleeder Swine

1972 ◽  
Vol 28 (03) ◽  
pp. 431-439
Author(s):  
C. N Cornell ◽  
R. G Cooper ◽  
M. E Muhrer ◽  
S Garb
Keyword(s):  
Beneficial Effect ◽  
Factor Viii ◽  
Bleeding Time ◽  
Platelet Concentrate ◽  
Platelet Adhesiveness ◽  
Normal Range ◽  
Von Willebrand's Disease ◽  
Von Willebrand’S Disease ◽  
Prolonged Bleeding Time ◽  
Platelet Infusion

SummaryBleeder swine are characterized by a prolonged bleeding time, decreased factor VIII levels or reduced platelet adhesiveness or both, and a factor VIII response to infusion of plasma or serum similar to that described in von Willebrand’s disease. In this study the factor VIII response of bleeder swine infused with fresh non-glass-contact pig plasma was similar to that reported in previous experiments after infusion of plasma that was frozen and stored for a variable period of time before use or was not free of glass contact. There was no beneficial effect on platelet adhesiveness. Following platelet concentrate infusion, there was no sustained normalization of platelet adhesiveness, although elevations into the normal range occurred at varying intervals after platelet infusion in two of three pigs investigated. Infusion of factor VIII-deficient plasma from a canine hemophiliac elicited a factor VIII response similar to that described in von Willebrand’s disease patients infused with plasma from factor VIII-deficient human hemophiliacs. In none of these experiments was there any apparent correction of the bleeding time.

1-Desamino-8-D-Arginine Vasopressin (DDAVP) Infusion in Type IIB von Willebrand’s Disease: Shortening of Bleeding Time and Induction of a Variable Pseudothrombocytopenia

1990 ◽  
Vol 64 (01) ◽  
pp. 117-120 ◽  
Author(s):  
Alessandra Casonato ◽  
M Teresa Sartori ◽  
Luigi de Marco ◽  
Antonio Girolami
Keyword(s):  
Monoclonal Antibody ◽  
Platelet Count ◽  
Arginine Vasopressin ◽  
Calcium Concentration ◽  
Sodium Citrate ◽  
Bleeding Time ◽  
Blood Collection ◽  
Von Willebrand's Disease ◽  
Blood Samples ◽  
Von Willebrand’S Disease

SummaryWe have investigated the effects of 1-desamino-8-D-arginine vasopressin (DDAVP) infusion on platelet count and bleeding time in 4 patients with type IIB von Willebrand’s disease (vWd). Three of four patients showed a normalization of the bleeding time within 1 h after the infusion, while bleeding time was not modified in the fourth. In accordance with the literature, thrombocytopenia was observed after DDAVP infusion, but this thrombocytopenia was due to the anticoagulants used for blood collection. In two patients (F. I., G. F.) no thrombocytopenia was observed when platelets were counted by fingerstick method but there was a 20% platelet decrease in blood samples collected in sodium citrate and a 50% decrease in samples collected in EDTA. Dramatic falls in platelet counts (70–95%) were observed in the additional two patients (C. A., D.Z.) after DDAVP infusion, when both sodium citrate or EDTA were used as anticoagulants. In the latter two patients there was also a 50% decrease in platelet count when the fingerstick method was used. The decrease in the patient’s platelet count in EDTA samples after DDAVP infusion could be prevented, in part, by the previous additions of an anti GPIb monoclonal antibody and an anti GPIIb-IIIa monoclonal antibody.Thus, the thrombocytopenia observed in the four IIB vWd patients studied after DDAVP infusion seems to be, at least partially, a pseudothrombocytopenia depending on the calcium concentration in the blood samples and the availability of GPIb and GPIIb-IIIa receptors. These findings and the normalization of the bleeding time observed in three of the four patients has led us to reconsider the possible use of DDAVP in the treatment of our IIB vWd patients.

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