RESUMEN
INTRODUCTION: Factor XIII (FXIII) deficiency is a rare hemorrhagic disorder whose early diagnosis is crucial for appropriate treatment and prophylactic supplementation in cases of severe deficiency. International guidelines recommend a quantitative FXIII activity assay as first-line screening test. FXIII antigen measurement may be performed to establish the subtype of FXIII deficiency (FXIIID) when activity is decreased. METHODS: The aim of this multicenter study was to evaluate the analytical and diagnostic levels of performance of a new latex immunoassay, K-Assay® FXIII reagent from Stago, for first-line measurement of FXIII antigen. Results were compared to those obtained with the Berichrom® FXIII chromogenic assay for measurement of FXIII activity. Of the 147 patient plasma samples, 138 were selected for analysis. RESULTS: The accuracy was very good, with intercenter reproducibility close to 7%. Five groups were defined on FXIII activity level (<5% (n = 5), 5%-30% (n = 23), 30%-60% (n = 17), 60%-120% (n = 69), above 120% (n = 24)), without statistical differences between activity and antigen levels (P value >0.05). Correlation of the K-Assay® with the Berichrom® FXIII activity results was excellent (r = 0.919). Good agreement was established by the Bland and Altman method, with a bias of +9.4% on all samples, and of -1.4% for FXIII levels lower than 30%. One patient with afibrinogenemia showed low levels of Berichrom® FXIII activity but normal antigen level and clot solubility as expected. CONCLUSIONS: The measurement of FXIII antigen using the K-Assay® is a reliable first-line tool for detection of FXIII deficiency when an activity assay is not available.
Asunto(s)
Deficiencia del Factor XIII/sangre , Factor XIII/análisis , Factor XIII/metabolismo , Femenino , Francia , Humanos , MasculinoAsunto(s)
Leucocitosis/mortalidad , Trombocitemia Esencial/mortalidad , Trombosis/mortalidad , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Estudios de Seguimiento , Hemoglobinas/análisis , Humanos , Leucocitosis/epidemiología , Leucocitosis/patología , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Factores de Riesgo , Tasa de Supervivencia , Trombocitemia Esencial/epidemiología , Trombocitemia Esencial/patología , Trombosis/epidemiología , Trombosis/patología , Adulto JovenRESUMEN
BACKGROUND AND OBJECTIVES: Inherited factor VII (FVII) deficiency is a rare bleeding disorder characterized by a poor relationship between reported FVII clotting activity (FVII:C) and bleeding tendency. Our study was aimed at defining biological parameters that are possibly predictive for bleeding risk in this condition. DESIGN AND METHODS: Forty-two FVII-deficient patients (FVII:C <30%) were classified into two opposite clinical groups defined as severe and non-or-mild bleeders. For each patient, plasma samples were collected and then investigated for FVII:C (using a sensitive method and human recombinant thromboplastin as the reagent), FVII antigen, activated FVII coagulant activity (FVIIa:C) and the free-form of tissue factor pathway inhibitor. RESULTS: None of these tests could be used as highly accurate predictors of bleeding. Nevertheless, both FVII:C and FVIIa:C differed significantly between the two clinical groups. Using ROC-curve analysis, two critical values of 8% and 3mIU/mL for FVII:C and FVIIa:C, respectively, could be proposed to discriminate between severe bleeders and non-or-mild bleeders. INTERPRETATION AND CONCLUSIONS: A highly accurate diagnostic test for predicting bleeding tendency in inherited FVII deficiency still eludes definition, highlighting the fact that factors other than FVII itself interfere with the expression of bleeding phenotypes in this condition. Nevertheless, potential critical values using sensitive FVII:C and FVIIa:C methods may be useful in clinical laboratories for FVII-deficient patients. Those patients with FVII:C levels higher than 8% FVII:C or FVIIa:C higher than 3 mIU/mL, with no other hemostatic defect, seem to have a minimal risk of severe bleeding. Extended clinical studies are needed to support these findings.
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Deficiencia del Factor VII/diagnóstico , Factor VII/análisis , Adolescente , Adulto , Trastornos de la Coagulación Sanguínea Heredados , Niño , Preescolar , Deficiencia del Factor VII/sangre , Femenino , Hemorragia/sangre , Humanos , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Riesgo , Índice de Severidad de la EnfermedadRESUMEN
In order to clarify the coagulation profile accompanying ischemic stroke, which may have implications on therapeutic strategies, we performed a prospective study to evaluate the hemostatic parameters in the first 24 h after the onset of cortical atherothrombotic infarct and lacunar infarction. Twenty-seven patients with cortical atherothrombotic infarction and 27 patients with lacunar infarction, diagnosed on clinical and CT-scan criteria, had blood samples taken within the first 24 h after onset of the stroke, and before anticoagulant treatment had been started. Levels of fibrinogen, von Willebrand factor, D-dimers, prothrombin factors 1 + 2, anti-thrombin III, and C-protein and S-proteins, were measured. Laboratory tests detected the following abnormalities: a protein C deficiency was observed in 1 case of cortical infarction and in 1 case of lacunar infarction; a decrease in S-protein was observed in 1 case of cortical infarction, and the presence of lupus anticoagulant in 4 cases (2 in cortical and 2 in lacunar infarction). Various degrees of coagulation activation were observed. Statistically significant activation of the coagulation was observed in the patients with cortical infarction, compared to normal patients adjusted for age: the levels of DDI were significantly raised (2298 +/- 2221 ng ml-1 vs. 750 +/- 400 ng ml-1) (p < 0.03) as were F1 + 2 levels (3.9 +/- 2.8 nmol l-1 vs. 1.5 +/- 0.9 nmol l-1). (p < 0.01). In the lacunar infarction group, there was a significant rise in F1 + 2 compared with normal patients adjusted for age (2.2 +/- 1.7 nmol l-1 vs. 1.5 +/- 0.9 nmol l-1) (p < 0.01), while the DDI level was in the normal range, when age was taken into account. In the cortical infarction group, we observed a significantly raised fibrinogen level (4.8 +/- 1.7 g l-1 vs. 3.7 +/- 1.0 g l-1) (p < 0.05) and von Willebrand factor level (271 +/- 104% vs. 178 +/- 103%) (p < 0.01) compared to the lacunar infarction group. In addition, we observed a significantly low level of S-protein in the cortical infarction group (105 +/- 29%) compared to the lacunar infarction group (127 +/- 28%) (p < 0.01). Confirmation of the role of enhanced thrombin activity in the pathogenesis of acute stroke may be an important determinant in its therapeutic management.
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Isquemia Encefálica/etiología , Infarto Cerebral/etiología , Trastornos Cerebrovasculares/etiología , Demencia por Múltiples Infartos/etiología , Trombosis/complicaciones , Anciano , Anciano de 80 o más Años , Pruebas de Coagulación Sanguínea , Trastornos Cerebrovasculares/clasificación , Femenino , Hemostasis , Humanos , Masculino , Persona de Mediana Edad , Estudios ProspectivosRESUMEN
Treatment by low density lipoprotein (LDL) apheresis using dextran sulfate columns (DSC) leads to hemostasis alterations with prolonged activated partial thromboplastin time (APTT) of more than 120 seconds. In order to explain this hypocoagulability, we studied hemostasis parameters both in patients and in the extracorporeal circulation (ECC). Hemostasis changes are first related to unfractionated heparin (UFH)--needed to avoid circuit coagulation--which leads to high residual heparinemia in the patient (more than 3 times the recommended level for therapeutic use). Second, the hypocoagulability is induced by a coagulation factor decrease (primarily factors V, VIII, and X) mainly due to an adsorption mechanism on dextran sulfate. Studies on samples from column inflow, outflow, and eluate confirm this mechanism. Low molecular weight heparin (LMWH) can be used in LDL apheresis on DSC without major changes in lipid removal or coagulation factors compared to UFH. The benefit of using LMWH is to reduce residual heparinemia into the therapeutic range.
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Eliminación de Componentes Sanguíneos/métodos , Circulación Extracorporea , Hemostasis , Hiperlipoproteinemia Tipo II/terapia , Lipoproteínas LDL/sangre , Nadroparina , Adulto , Factores de Coagulación Sanguínea/análisis , Eliminación de Componentes Sanguíneos/instrumentación , Sulfato de Dextran , Humanos , Hiperlipoproteinemia Tipo II/sangre , Persona de Mediana EdadRESUMEN
The heparinization of multiple trauma patients during hemofiltration involves a high risk of bleeding. We report here two continuous hemofiltration cases -15 and 60 days in duration-using a low molecular weight heparin, enoxaparine. No major bleeding or clotting was observed. However, continuous infusion of the heparin is needed and the rate of delivery of the hemofiltration pump must be taken into consideration since it influences the clearance of the drug through the hemofiltration membrane. Anti-Xa activity measurement is not sufficient to adjust the heparin dose.
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Hemofiltración , Heparina de Bajo-Peso-Molecular/administración & dosificación , Lesión Renal Aguda/tratamiento farmacológico , Adulto , Heparina de Bajo-Peso-Molecular/uso terapéutico , Humanos , Masculino , Traumatismo Múltiple/terapiaRESUMEN
Usual coagulation tests--Platelet count, Prothrombin Time (PT), Activated Partial Thromboplastine Time (APTT)--detect most of coagulation abnormalities in preoperative situation. Emergency surgery remains possible without bleeding in most cases. Patient history of haemostatic responses is absolutely necessary to know in addition to coagulation tests. Platelets transfusion may often be avoid in thrombocytopenic patients with platelet count higher than 50,000 mm3. The PT and/or APTT perturbations need further laboratory investigations to estimate the bleeding risk which is not constant. The correction of an antithrombotic treatment is usually easy except for drugs which inhibit platelet aggregation.
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Pruebas de Coagulación Sanguínea , Recuento de Plaquetas , Anticoagulantes/uso terapéutico , Trastornos de la Coagulación Sanguínea/diagnóstico , Urgencias Médicas , Humanos , Inmunoglobulinas/análisis , Cuidados Preoperatorios , Vitamina K/antagonistas & inhibidoresRESUMEN
The effects of the nitrovasodilator Sin-1, which is thought to work through cyclic-GMP, were studied using human, aequorin-loaded, washed platelets. Changes in light transmission (shape change, aggregation), and in intracellular calcium were simultaneously recorded. Evidence was obtained for an inhibitory effect distal to calcium changes, in a similar way than a prostacyclin analogue, Iloprost. By contrast the response to an activator of protein kinase C was unaffected. There was a partial, parallel decrease in calcium changes and aggregation induced with thrombin, and a total inhibition of the responses to ADP (linked to a calcium influx).
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Molsidomina/análogos & derivados , Inhibidores de Agregación Plaquetaria , Aequorina , Humanos , Técnicas In Vitro , Molsidomina/farmacologíaRESUMEN
Hepatic venocclusive disease (VOD) is a frequent complication of bone marrow transplantation (BMT). Analysis of 13 cases observed during a 3-year period in our BMT center shows that VOD is associated with a constant peripheral thrombocytopenia and refractoriness to platelet transfusion. These signs appear in the very early stage of VOD, five to ten days before the classical signs, painful hepatomegaly and sudden weight gain. Analysis of platelet consumption, frequency of platelet transfusion and platelet recovery, and examination of known causes of peripheral thrombocytopenia (mainly allo- and autoimmunization, disseminated intravascular coagulation [DIC] and splenomegaly) lead to the conclusions that this association is not coincidental. The exact mechanism of platelet consumption in VOD is unknown.