thromboplastin and von-Willebrand-Diseases

Hemostasis and thrombosis are now increasingly recognized as integrally related to blood rheology and blood flow. Platelets, for example, are known to access the vessel wall in ways which depend upon the small-scale motions of neighboring erythrocytes, and access one another via collisions driven by gradients in blood flow velocity. In this context, flow devices have become a subject of great interest in the clinical assessment of bleeding disorders, especially platelet function defects and von Willebrand disease. While these devices currently lack standardization and outcomes measures which establish clear clinical utility, their promise remains great, particularly in the potential to simulate the microenvironment of arteries vs. veins and in their ability to incorporate such intrinsically flow-dependent phenomena as co-localization of tissue-factor-bearing microparticles with platelets, the weakness of the GPIb-vWF bond at very high shear stresses, and even the hemostatic and antithrombotic function of vascular endothelium. In contrast, currently utilized assays are often performed under static conditions that do not involve flow and therefore are not able to simulate the microenvironment of arteries and veins.

Topics: Animals; Blood Flow Velocity; Blood Platelets; Blood Vessels; Cell-Derived Microparticles; Endothelium, Vascular; Erythrocytes; Hemorheology; Hemorrhage; Hemostasis; Humans; Membrane Glycoproteins; Platelet Glycoprotein GPIb-IX Complex; Thromboplastin; Thrombosis; von Willebrand Diseases; von Willebrand Factor

Topics: Antigen-Antibody Complex; Biomarkers; Hemophilia A; Humans; Immunoassay; Partial Thromboplastin Time; Reference Values; Specimen Handling; Thromboplastin; von Willebrand Diseases

Topics: Angioedema; Animals; Antithrombins; Blood Coagulation; Blood Coagulation Factors; Blood Platelets; Complement System Proteins; Enzyme Precursors; Factor VIII; Factor XII; Female; Fibrin; Fibrinogen; Fibrinolysis; Hemophilia A; Hemostasis; Humans; Kallikreins; Male; Platelet Adhesiveness; Thrombin; Thromboplastin; Thrombosis; von Willebrand Diseases

Topics: Adult; Blood Coagulation Disorders; Blood Coagulation Tests; Blood Platelet Disorders; Child; Factor X; Factor XII; Female; Fibrinolysin; Fibrinolysis; Hemostasis; Humans; Lipoproteins; Male; Prothrombin Time; Purpura, Thrombocytopenic; Thrombin; Thromboplastin; Thrombosis; von Willebrand Diseases

Topics: Blood Coagulation; Blood Coagulation Factors; Factor IX; Factor V; Factor VII; Factor VIII; Factor X; Factor XI; Factor XII; Factor XIII; Fibrin; Fibrinogen; Humans; Phosphatidylethanolamines; Prothrombin; Thromboplastin; von Willebrand Diseases

Topics: Adrenal Cortex Hormones; Antibodies; Calcium; Chromosomes; Epinephrine; Factor IX; Factor VIII; Hemophilia A; Humans; Phospholipids; Progesterone; Spleen; Thromboplastin; Thyroxine; von Willebrand Diseases

Topics: Animals; Aortic Valve Stenosis; Aortitis; Calcinosis; Humans; Osteoprotegerin; RANK Ligand; Thromboplastin; von Willebrand Diseases

We report a family with a combined factor VII Padua defect and von Willebrand's disease (vWd). The propositus is a 9-year-old child with a moderate bleeding tendency who appeared to be heterozygous for both factor VII Padua and type I vWd. The diagnosis of factor VII Padua was based on a normal factor VII antigen and factor VII activity which was low with rabbit brain thromboplastin but normal with ox brain thromboplastin. Type I vWd was diagnosed because of a concomitant decrease of von Willebrand factor antigen (vWf:Ag) and vWf ristocetin-cofactor activity (vWf:RCoF), associated with the presence of vWf multimers of all sizes in plasma and platelets. The parents were not consanguineous but came from the same isolated river Piave valley in North Eastern Italy where the factor VII Padua defect was first described. The father had the factor VII Padua defect but was clinically asymptomatic in accordance with the heterozygous state. The propositus's mother had type I vWd and was mildly symptomatic. The propositus' sisters, who were clinically asymptomatic, were both heterozygotes for factor VII Padua. The infusion of DDAVP normalized the factor VIII/vWf pattern in all patients. In the propositus, in contrast to the mother and normal subjects, showed a more rapid clearance both of vWf and factor VIII. The same pattern, albeit to a lesser degree, was also observed in the father.

Topics: Child; Deamino Arginine Vasopressin; Factor VII; Factor VII Deficiency; Female; Humans; Italy; Male; Pedigree; Thromboplastin; von Willebrand Diseases

Topics: Animals; Blood Coagulation Disorders; Brain Chemistry; Cattle; DNA Mutational Analysis; Factor VII; Factor VII Deficiency; Humans; Thromboplastin; von Willebrand Diseases

FVIII/VWF in plasma and platelets was studied by various methods in 16 patients with von Willebrand's disease. These methods included measurements of both VIIIR:Ag and VIIIR:RCo levels, radio-crossed immunoelectrophoresis, analysis of the dose-response curves with both fluid-phase and two-site immunoradiometric assays, and SDS-agarose-acrylamide gel electrophoresis. Studies of normal plasma and platelets by the last-named method disclosed the presence of nine to 10 clearly resolvable bands with molecular weights of approximately 1 to 10 X 10(6) and unresolved higher-molecular-weight material, consistent with the previously described multimeric structure of FVIII/VWF. The multimeric structure and antigenic reactivity of FVIII/VWF were normal in 11 patients with type I von Willebrand's disease. However, measurements of the VIIIR:Ag content in plasma and platelets disclosed the presence of three subgroups. In one (type I-1), the VIIIR:Ag content of both plasma and platelets was decreased; in type I-2, VIIIR:Ag was decreased in plasma but normal in platelets, whereas the reverse was found in two patients with type I-3. In five patients with type IIA von Willebrand's disease we observed various abnormalities in the multimeric structure and antigenic reactivity of FVIII/VWF. The distinguishing features in two patients, designated type IIA-1 and IIA-2, were a decreased amount of high-molecular-weight FVIII/VWF and an impaired antigenic reactivity in both plasma and platelets; the defects in type IIA-2 were qualitatively different and more strikingly abnormal than those in type IIA-1. In three other patients, designated type IIIA-3, a less severe deficit of high-molecular-weight FVIII/VWF in plasma was observed and, in addition, the multimeric structure of FVIII/VWF in platelets was normal. The findings in this study demonstrate that a variety of defects in the synthesis, release, antigenic reactivity, and multimerization of FVIII/VWF are probably responsible for the heterogeneous findings in patients with von Willebrand's disease.

Topics: Adult; Blood Coagulation Factors; Electrophoresis, Polyacrylamide Gel; Epitopes; Factor VIII; Female; Humans; Immunoelectrophoresis, Two-Dimensional; Macromolecular Substances; Male; Middle Aged; Molecular Weight; Thromboplastin; von Willebrand Diseases; von Willebrand Factor

Topics: Adolescent; Adult; Blood Coagulation Tests; Child; Child, Preschool; Factor VIII; Female; Hemarthrosis; Hematoma; Hemophilia A; Hemophilia B; Humans; Infant; Male; Oral Hemorrhage; Thromboplastin; von Willebrand Diseases

Topics: Afibrinogenemia; Blood Coagulation Disorders; Blood Coagulation Tests; Factor V Deficiency; Factor VII Deficiency; Factor X Deficiency; Factor XI Deficiency; Factor XII Deficiency; Factor XIII Deficiency; Hemophilia A; Hemophilia B; Humans; Hypoprothrombinemias; Kaolin; Phosphatidylethanolamines; Prekallikrein; Prothrombin Time; Thrombin; Thromboplastin; von Willebrand Diseases

The influence of different temperatures between 13 degrees C and 45 degrees C on coagulation factors in vitro was studied by measuring clotting time with the recalcification time, partial thromboplastin time (PTT), and thromboplastin time test. In all three tests the shortest clotting times were measured at a temperature of 40 degrees C. The relation between temperature and clotting time was similar in fresh plasma and in plasma which had been stored at a temperature of --20 degrees C before examination. However, in all tests stored plasma showed shorter coagulation times. Prolongation of coagulation time at 45 degrees C is caused by irreversible reduction of coagulation activity in the plasma. At the same time thromboplastin- and PTT-reagent are imparied in their coagulation acitvity by a temperature of 45 decrees C. In comparison to plasma obtained from healthy persons plasma from patients with hemophilia A or B or with v. Willebrand's disease reacted more sensitive to changes in temperature in the PTT test. The coagulation defect was definitely more pronounced at 27 degrees and 17 degrees C than at 37 degrees C. It was not possible to differentiate these three coagulopathies with the PTT test at 27 degrees and 17 degrees C.

Topics: Blood Coagulation; Blood Coagulation Tests; Freezing; Hemophilia A; Hemophilia B; Humans; Temperature; Thromboplastin; von Willebrand Diseases

Reagents may be prepared from normal plasma and used with the prothrombin time and partial thromboplastin time tests to distinguish isolated defects of factors I, II, VII, VIII, IX, X, XI, or XII.

Topics: Adolescent; Adult; Aluminum Hydroxide; Blood Coagulation Disorders; Blood Coagulation Tests; Child; Child, Preschool; Factor XI Deficiency; Female; Hemophilia A; Hemophilia B; Humans; In Vitro Techniques; Kaolin; Male; Middle Aged; Phospholipids; Plasma; Prothrombin Time; Thromboplastin; Tromethamine; von Willebrand Diseases

Topics: Adult; Afibrinogenemia; Animals; Blood Cell Count; Blood Coagulation; Blood Coagulation Tests; Blood Platelets; Cattle; Citrates; Collagen; Crystallization; Dogs; Edetic Acid; Factor VII Deficiency; Factor X Deficiency; Factor XII; Female; Fibrinogen; Hemophilia A; Hemostasis; Heparin; Humans; Male; Platelet Adhesiveness; Platelet Aggregation; Purpura, Thrombocytopenic; Thrombin; Thromboplastin; von Willebrand Diseases

Topics: Adenosine Diphosphate; Antigen-Antibody Reactions; Blood Coagulation; Blood Platelets; Blood Transfusion; Chromosome Aberrations; Chromosome Disorders; Cryoglobulins; Diagnosis, Differential; Factor VIII; Female; Hemophilia A; Humans; Male; Models, Biological; Platelet Adhesiveness; Serotonin; Sex Chromosome Aberrations; Thromboplastin; von Willebrand Diseases

Topics: Adolescent; Adult; Blood Coagulation Disorders; Child, Preschool; Culture Techniques; Factor VII; Factor X; Factor XI; Factor XI Deficiency; Factor XII; Female; Fibroblasts; Hemophilia A; Hemophilia B; Humans; Male; Middle Aged; Skin; Thromboplastin; von Willebrand Diseases

Topics: Blood Coagulation Tests; Heparin; Humans; In Vitro Techniques; Male; Thrombin; Thromboplastin; Time Factors; von Willebrand Diseases; Warfarin

Topics: Blood Cell Count; Blood Coagulation; Blood Coagulation Disorders; Blood Coagulation Factors; Blood Platelet Disorders; Blood Platelets; Diagnosis, Differential; Disseminated Intravascular Coagulation; Female; Hemoglobinometry; Hemophilia A; Hemophilia B; Hemorrhage; Humans; Male; Prothrombin Time; Purpura; Purpura, Thrombocytopenic; Purpura, Thrombotic Thrombocytopenic; Rheumatic Diseases; Telangiectasia, Hereditary Hemorrhagic; Thromboplastin; von Willebrand Diseases

Topics: Anticoagulants; Blood Coagulation Disorders; Consanguinity; Enzyme Precursors; Factor IX; Factor VIII; Hemophilia A; Humans; Pedigree; Thromboplastin; von Willebrand Diseases

Topics: Child; Factor IX; Factor VIII; Hemostatics; Humans; Male; Oral Hemorrhage; Preoperative Care; Thromboplastin; Tooth Extraction; Venoms; von Willebrand Diseases

Topics: Adult; Aerospace Medicine; Blood Cell Count; Blood Coagulation Tests; Blood Platelets; Blood Sedimentation; Eosinophils; Fibrinolysis; Hematocrit; Hemoglobinometry; Humans; Lymphocytes; Male; Monocytes; Prognosis; Prothrombin Time; Reticulocytes; Thromboplastin; von Willebrand Diseases

Topics: Blood Coagulation Tests; Diagnosis, Differential; Factor IX; Factor VIII; Hemophilia A; Hemophilia B; Humans; Male; Methods; Prothrombin Time; Thromboplastin; von Willebrand Diseases

Topics: Child; Child, Preschool; Factor VIII; Female; Hematoma; Hemophilia A; Hemophilia B; Hemorrhage; Humans; Infant; Infant, Newborn; Italy; Male; Thromboplastin; von Willebrand Diseases

Topics: Adenine Nucleotides; Blood Coagulation; Blood Coagulation Disorders; Blood Platelets; Citrates; Densitometry; Factor XII; Freezing; Hemorrhagic Disorders; Humans; Silicones; Thrombin; Thromboplastin; von Willebrand Diseases

Topics: Blood Coagulation Tests; Blood Platelet Disorders; Blood Transfusion; Factor VIII; Gastrointestinal Hemorrhage; Hemophilia A; Humans; Male; Thromboplastin; von Willebrand Diseases

Topics: Blood Coagulation Tests; Blood Platelet Disorders; Blood Transfusion; Factor VIII; Genetics, Medical; Hemophilia A; Hot Temperature; Humans; Hydrogen-Ion Concentration; Refrigeration; Thromboplastin; von Willebrand Diseases

Topics: Blood Coagulation Tests; Delivery, Obstetric; Female; Hemophilia B; Humans; Pathology; Postpartum Period; Pregnancy; Puerperal Disorders; Thromboplastin; von Willebrand Diseases

Topics: Adolescent; Blood Coagulation Tests; Hemophilia A; Humans; Research; Thromboplastin; von Willebrand Diseases

Topics: Globulins; Hemophilia A; Serum Globulins; Thromboplastin; von Willebrand Diseases

Topics: Blood Coagulation Disorders; Hemophilia A; Humans; Thrombocytopenia; Thromboplastin; Thrombosis; von Willebrand Diseases

Topics: Adsorption; Blood Coagulation; Factor VIII; Globulins; Hemophilia A; Humans; Thromboplastin; von Willebrand Diseases

Topics: Blood Coagulation; Globulins; Hemophilia A; Plasma; Thromboplastin; von Willebrand Diseases

Topics: Anticoagulants; Blood Coagulation; Hemophilia A; Humans; Thromboplastin; von Willebrand Diseases

Topics: Hemophilia A; Humans; Syndrome; Thromboplastin; von Willebrand Diseases

Topics: Blood Coagulation; Blood Platelets; Hemophilia A; Humans; Thromboplastin; von Willebrand Diseases

Topics: Hemophilia A; Humans; Thromboplastin; von Willebrand Diseases

Topics: Hemophilia A; Hemophilia B; Humans; Medicine; Sex Chromosome Disorders; Thromboplastin; von Willebrand Diseases

Topics: Hemophilia A; Hemophilia B; Thromboplastin; von Willebrand Diseases

Topics: Blood Coagulation Disorders; Blood Platelets; Hemophilia A; Humans; Plasma; Thromboplastin; von Willebrand Diseases