scholarly journals Circulating anticoagulant in a family with prolonged bleeding time and factor VIII deficiency

Blood ◽  
1977 ◽  
Vol 49 (5) ◽  
pp. 799-806
Author(s):  
M Diez-Ewald ◽  
EC Lian ◽  
R Nunez ◽  
D Deykin ◽  
DR Harkness
Keyword(s):  
Platelet Aggregation ◽  
Factor Viii ◽  
Bleeding Time ◽  
Factor Viii Activity ◽  
Clot Retraction ◽  
Factor Viii Related Antigen ◽  
Factor Viii Deficiency ◽  
Prolonged Bleeding ◽  
Prolonged Bleeding Time ◽  
Aggregation Activity

A circulating anticoagulant against factor VIII activity was demonstrated in the plasma of a boy from a family with both factor VIII deficiency and prolonged bleeding time. However, the factor VIII- related antigen, ristocetin-induced platelet aggregation activity, platelet retention in glass bead columns, platelet aggregation with adenosine 5′-diphosphate, collagen and epinephrine, and clot retraction among affected members were normal. The electrophoretic mobility of factor VIII-related antigen on crossed immunoelectrophoresis was normal. The inactivation of factor VIII activity by the inhibitor was time dependent and was nonlinear as the concentration of the inhibitor was increased. Immunotyping showed that the inhibitor was IgG with k light chains.

Circulating anticoagulant in a family with prolonged bleeding time and factor VIII deficiency

Blood ◽  
1977 ◽  
Vol 49 (5) ◽  
pp. 799-806 ◽  
Author(s):  
M Diez-Ewald ◽  
EC Lian ◽  
R Nunez ◽  
D Deykin ◽  
DR Harkness
Keyword(s):  
Platelet Aggregation ◽  
Factor Viii ◽  
Bleeding Time ◽  
Factor Viii Activity ◽  
Clot Retraction ◽  
Factor Viii Related Antigen ◽  
Factor Viii Deficiency ◽  
Prolonged Bleeding ◽  
Prolonged Bleeding Time ◽  
Aggregation Activity

Abstract A circulating anticoagulant against factor VIII activity was demonstrated in the plasma of a boy from a family with both factor VIII deficiency and prolonged bleeding time. However, the factor VIII- related antigen, ristocetin-induced platelet aggregation activity, platelet retention in glass bead columns, platelet aggregation with adenosine 5′-diphosphate, collagen and epinephrine, and clot retraction among affected members were normal. The electrophoretic mobility of factor VIII-related antigen on crossed immunoelectrophoresis was normal. The inactivation of factor VIII activity by the inhibitor was time dependent and was nonlinear as the concentration of the inhibitor was increased. Immunotyping showed that the inhibitor was IgG with k light chains.

Essential Athrombia

1975 ◽  
Vol 33 (02) ◽  
pp. 278-285 ◽  
Author(s):  
Şeref Inceman ◽  
Yücel Tangün
Keyword(s):  
Platelet Aggregation ◽  
Bleeding Time ◽  
Bleeding Tendency ◽  
Clot Retraction ◽  
Platelet Factor ◽  
Prolonged Bleeding Time ◽  
Normal Platelet ◽  
Aggregation Response ◽  
Platelet Disorder ◽  
Fibrinogen Content

SummaryA constitutional platelet function disorder in a twelve-year-old girl characterized by a lifelong bleeding tendency, prolonged bleeding time, normal platelet count, normal clot retraction, normal platelet factor 3 activity and impaired platelet aggregation was reported.Platelet aggregation, studied turbidimetrically, was absent in the presence of usual doses of ADP (1–4 μM), although a small wave of primary aggregation was obtained by very large ADP concentrations (25–50 μM). The platelets were also unresponsive to epinephrine, thrombin and diluted collagen suspensions. But an almost normal aggregation response occurred with strong collagen suspensions. The platelets responded to Ristocetin. Pelease of platelet ADP was found to be normal by collagen and thrombin, but impaired by kaolin. Platelet fibrinogen content was normal.The present case, investigated with recent methods, confirms the existence of a type of primary functional platelet disorder characterized solely by an aggregation defect, described in 1955 and 1962 under the name of “essential athrombia.”

Increased Ristocetin Induced Binding of Factor VIII to Platelets in Von Willebrand’s Disease (vWd)

1979 ◽  
Author(s):  
Z.M. Ruggeri ◽  
F.I. Pareti ◽  
P.M. Mannucci ◽  
T.S. Zimmerman
Keyword(s):  
Platelet Aggregation ◽  
Factor Viii ◽  
Bleeding Time ◽  
Human Platelet ◽  
Bleeding Tendency ◽  
Platelet Factor ◽  
Induce Platelet Aggregation ◽  
Prolonged Bleeding Time ◽  
Crossed Immunoelectrophoresis ◽  
Ristocetin Cofactor

Initial reports of ristocetin-induced platelet aggregation (RIPA) demonstrated it to be decreased in some patients with vWd. We now report 20 patients (from five unrelated families) in whom RIP A was increased, apparently as the result of an increased ristocetin-induced binding of Factor VIIIrelated antigen (VIIIR:Ag) to platelets. All the patients had a life-long bleeding tendency, with prolonged bleeding time, and an abnormal two-dimensional crossed immunoelectrophoresis (2DCIE). Increased RIPA was demonstrated by measuring the minimum ristocetin concentration necessary to induce platelet aggregation. This was 0.42 mg/ml á 0.11 SD in the patients, and 0.91 á 0.097 SD in 17 normals (t = 13.83; P < 0.001). VIIIR:Ag binding to platelets occurred at ristocetin concentrations (0.4 mg/mI) which were ineffective in normals (who required >0.6 mg/mI). In contrast, the VIIIR:Ag of other patients with abnormal 2DCIE and markedly decreased RIP A did not bind to platelets at ristocetin concentrations as high as I mg/ml. It has been previously demonstrated that 30% to 60% of normal VIIIR:Ag binds to isolated human platelet membranes in the absence of ristocetin or any other agent, and that binding is restricted to the larger forms of VIIIR:Ag. However, VIIIR:Ag from the patients with increased RIPA, including two with normal ristocetin cofactor activity, showed decreased or undetectable binding as did all other patients with abnormal 2DCIE. This study suggests that ristocetin induced platelet Factor VIII interaction does not accurately reflect the “bleeding time factor” defect in vWd.

Studies on a Variant of Factor VIII Resulting in ’von Willebrand’s Disease»

1975 ◽  
Author(s):  
F. G. H. Hill ◽  
M. C. K. Chan ◽  
R. M. Hardisty
Keyword(s):  
Factor Viii ◽  
Bleeding Time ◽  
Von Willebrand's Disease ◽  
Von Willebrand’S Disease ◽  
Prolonged Bleeding ◽  
Prolonged Bleeding Time

A variant of von Willebrand’s disease in a 14-year-old girl is described, characterised by a prolonged bleeding time and defective ristocetin aggregation (VIIIWF 6%), with VIIIRAg 70-110% and VIIIC 40-60%. The electrophorotic mobility of her VIIIRAg in agarose at pH 9.2 was intermediate between normal VIIIRAg and that of the patient of Kernoff et al, (1), and identical with that of Case 4 of Peake et al. (2). Further characteristics of the factor VIII molecule in this patient’s plasma and platelets will be presented, including antigenic, physicochemical and functional propeertis.1. Kernoff, P. B. A. et al. (1974). Brit. J. Haemat. 26, 435.2. Peake, I. R. et al. (1974). N. Engl. J. Med. 291, 113.

Sustained Bleeding after a leech Bite in the Apparent Absence of Hirudin

1989 ◽  
Vol 61 (03) ◽  
pp. 366-369 ◽  
Author(s):  
R Munro ◽  
F O P Hechtel ◽  
R T Sawyer
Keyword(s):  
Platelet Aggregation ◽  
Bleeding Time ◽  
Medicinal Leech ◽  
Bite Wound ◽  
Apparent Absence ◽  
Coagulation Parameters ◽  
Prolonged Bleeding ◽  
Prolonged Bleeding Time ◽  
Human Volunteers

SummaryThe bite of the medicinal leech bleeds for many hours. For decades it has been assumed that the remarkably prolonged bleeding time of a leech bite wound is due to hirudin, a specific anti-thrombin secreted by the leech during feeding. By measuring haematological parameters of blood oozing from a leech bite wound on 15 different occasions in 7 human volunteers, we demonstrate that the hirudin-sensitive coagulation parameters, including thrombin-induced platelet aggregation, are prolonged for only 15 min, after which they return to normal. This suggests that excess hirudin secreted by the leech is washed out during this period. However, bleeding from the leech bite wound persists for a mean of 10 h. Platelets in smears of exuding blood show no evidence of spontaneous aggregation, but in vitro platelet aggregation can be induced by exogenous collagen at any time. In view of sustained bleeding in the apparent absence of hirudin, attention is focussed onto an unsuspected factor or factors which may better explain the prolonged bleeding phenomenon.

Platelet Function in Congenital Afibrinogenemia

1965 ◽  
Vol 14 (03/04) ◽  
pp. 361-373 ◽  
Author(s):  
E Gugler ◽  
E. F Lüscher
Keyword(s):  
Platelet Aggregation ◽  
Bleeding Time ◽  
Clot Retraction ◽  
Prolonged Bleeding Time ◽  
Congenital Afibrinogenemia ◽  
Fraction I ◽  
Glass Surfaces ◽  
Platelet Functions

SummaryPlatelet functions have been studied in relation to hemostasis in two patients with congenital afibrinogenemia.Neither in the plasma nor in the aqueous platelet extracts of these patients was fibrinogen detectable by immunoelectrophoresis or with the aid of the Ouchterlony technique. ADP-induced platelet aggregation, adhesion to connective tissue particles, viscous metamorphosis under the influence of thrombin, clot retraction activity of the platelets, as well as their factor 3 activity were all found normal. Abnormal was the behaviour of the patient’s platelets on glass surfaces : they were unable to adhere to glass and the typical spreading on such surfaces was equally missing. This defect was normalized in vitro by the addition of small amounts of fibrinogen and correspondingly the patients platelets showed normal adhesiveness after fibrinogen transfusions. Normal platelets, suspended in afibrinogénémie plasma lost their adhesiveness toward glass surfaces.After transfusion of Cohn fraction I the prolonged bleeding time of the patients was normal and the clinical improvement presisted for a period of about 3 weeks, this inspite of the fact, that no fibrinogen was detectable by the usual methods 10 days after the transfusion.The significance of these results as well as their implications for the role of fibrinogen in hemostasis are discussed.

scholarly journals Disturbed platelet aggregation to collagen associated with an antibody against an 85- to 90-Kd platelet glycoprotein in a patient with prolonged bleeding time

Blood ◽  
1992 ◽  
Vol 79 (6) ◽  
pp. 1466-1471 ◽  
Author(s):  
H Deckmyn ◽  
E Van Houtte ◽  
J Vermylen
Keyword(s):  
Platelet Aggregation ◽  
Bleeding Time ◽  
Polyacrylamide Gel Electrophoresis ◽  
Sodium Dodecyl ◽  
Platelet Glycoprotein ◽  
Two Dimensional ◽  
Membrane Glycoproteins ◽  
Prolonged Bleeding ◽  
Prolonged Bleeding Time ◽  
Normal Platelet

We studied a 5-year-old girl presenting with a markedly prolonged bleeding time. Her platelets were refractory to collagen stimulation, but the response to other agonists was normal. There were no coagulation abnormalities as measured by standard tests. Two- dimensional electrophoresis showed no abnormalities of the patient's platelet membrane glycoproteins. When the patient's plasma or purified plasma IgG was mixed with normal platelets, collagen-induced platelet aggregation was blocked. Western blotting showed the presence of an antibody in the patient's plasma directed against a protein of molecular weight 85 to 90 Kd under both reducing and nonreducing conditions. This protein comigrated with glycoprotein (GP) IV immunoprecipitated by OKM5 from 125I-labeled platelets. Immunoprecipitation of 125I-labeled normal platelet glycoproteins with the patient's IgGs also yielded an 85- to 90-Kd protein that migrated on the diagonal following nonreduced/reduced two-dimensional sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE). Despite similarities in electrophoretic behavior, the antigen was not demonstrated to be GPIV, since purified GPIV was not recognized by the antibody.

Thrombocytopathy Induced by Acquired Circulating Factor VIII Inhibitor

1975 ◽  
Author(s):  
M. Cortellaro ◽  
E. Pogliani ◽  
E. Cofrancesco ◽  
E. E. Polli
Keyword(s):  
Platelet Aggregation ◽  
Factor Viii ◽  
Bleeding Time ◽  
Steroid Treatment ◽  
Factor Viii Inhibitor ◽  
Factor Viii Activity ◽  
Antiplatelet Antibody ◽  
Normal Platelet ◽  
Viii Inhibitor ◽  
Willebrand Factor

A reduced ADP and ristocetin platelet aggregation by acquired circulating factor VIII inhibitor (2 U/ml) was found in a young woman with S. L. E. Bleeding time was prolonged, factor VIII activity decreased (25%), Willebrand antigen and Willebrand factor were normal. PF3 assay and PF4 release were normal. Platelet (14C) - serotonin uptake, but not release, was reduced. Antiplatelet antibody was not detected.Patient’s plasma inhibited the ristocetin and ADP induced platelet aggregation of normal PRP, but not of normal and patient GFP.After steroid treatment F. VIII inhibitor and thrombocytopathy disappeared.It is suggested that the circulating inhibitor is able to coat autologous and isologous unwashed platelets, interfering with platelet function.

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