LUPUS ANTICOAGULANT (LA) COEXISTENT WITH TRANSIENT PROTHROMBIN (FII) INHIBITOR: FTI DEFICIENCY DUE TO CLEARANCE OF THE B/MUNOCOMPLEX

1987 ◽  
Author(s):  
R Redaelli ◽  
F Baudo ◽  
B Busnach ◽  
T M Caimi ◽  
L Perrino ◽  
...  
Keyword(s):  
Survival Time ◽  
Replacement Therapy ◽  
Lupus Anticoagulant ◽  
Normal Tissue ◽  
Past History ◽  
Laboratory Data ◽  
Coagulation Factors ◽  
Precipitin Line ◽  
Coagulation Tests ◽  
Tissue Thromboplastin

23 y.o. man with acute nephritis and bleeding (epistaxis, ecchymosis) at presen-taticn. Family and personal past history negative for bleeding. Laboratory data consistent with SLE. Coagulation tests: FT Ratio (R) 1.8, aPTT R 2.4, FII:C <1%, FIIR:Ag 996, other coagulation factors normal. Tissue thromboplastin inhibition test (TTIT) R 2.8, congenital FII deficiency (696) R 1.6.1. FII survival time (Fll-ccncentrate infusion - 60 U/kg) t1/2: 9 hours.2. FII neutralizing activity (FTI:C normal plasma (NP) + buffer 5996; NP + patient plasna {PtP) 5096): absent.3. Irmunoccrplex formaticn4. FII inhibitor characterization (purified FII coupled to CNBr-activatedSepharose →PtP incubation with Fll-Sepharose→specific antiFII irrrrunoglobulins (Ig)* elution at acid pH→identification by double iimunodifftision): precipitin line with anti IgA, anti IgG2, anti k, anti 1.5. LA characterization (after FII inhibitor disappearance): TTTT on mixtures NP + PtP or N Ig in equal volumes.Diagnosis: SIE, LA (IgG); polyclonal (IgA, IgG2, k, 1) not neutralizing FII inhibitor; hypoprothrxmbinemia due to clearance of the irrrrunocorrplex.FII inhibitor was transient. Bleeding was rapidly controlled by replacement therapy. LA persits after FII inhibitor disappearance.

Detection of Lupus-Like Anticoagulants by an Enzyme-Linked Immunosorbent Assay Using a Partial Thromboplastin as Antigen; A Comparative Study

1990 ◽  
Vol 64 (01) ◽  
pp. 026-031 ◽  
Author(s):  
J Arnout ◽  
E Huybrechts ◽  
M Vanrusselt ◽  
C Falcon ◽  
J Vermylen
Keyword(s):  
Antiphospholipid Antibodies ◽  
Lupus Anticoagulant ◽  
Activated Partial Thromboplastin Time ◽  
The Other ◽  
Quantitative Detection ◽  
Enzyme Linked Immunosorbent Assay ◽  
Clotting Time ◽  
Coagulation Tests ◽  
Tissue Thromboplastin ◽  
Kaolin Clotting Time

SummaryClotting assays allow qualitative rather than quantitative detection of the lupus anticoagulant. We have therefore studied the usefulness of an ELISA using a commercial partial thromboplastin, Thrombofax, oS antigen; the results obtained on 146 selected patient plasmas were compared to the results of coagulation tests (kaolin clotting time, tissue thromboplastin inhibition test, activated partial thromboplastin time) and of ELISAs using cardiolipin or phosphatidylserine as antigen. While satisfactory agreement was found within the group of coagulation tests or that of ELISAs, only a moderate agreement was obtained between clotting tests and ELISAs, the best being with the partial thromboplastin ELISA using low plasma dilutions. The study further indicates that ELISA techniques cannot entirely replace coagulation tests for the detection of a lupus anticoagulant, even when a partial thromboplastin is used as antigen. On the other hand, coagulation tests are less sensitive than ELISAs for the detection of antiphospholipid antibodies.

Evaluation of Recently Described Tests for Detection of the Lupus Anticoagulant

1994 ◽  
Vol 72 (05) ◽  
pp. 728-733 ◽  
Author(s):  
Ricardo R Forastiero ◽  
Graciela S Cerrato ◽  
Luis O Carreras
Keyword(s):  
Human Placenta ◽  
Partial Thromboplastin Time ◽  
Lupus Anticoagulant ◽  
High Sensitivity ◽  
Activated Partial Thromboplastin Time ◽  
Lupus Anticoagulants ◽  
Coagulation Tests ◽  
Prolonged Aptt ◽  
Tissue Thromboplastin ◽  
Standard Tests

SummaryIt is known that lupus anticoagulants (LA) are antibodies which interfere with phospholipid-dependent coagulation tests, but due to the heterogeneity of LA and the differences in sensitivity of reagents and tests, the diagnosis of LA remains difficult.Recently, Triplett et al. (26) have proposed a new test based on two venoms, Textarin (T) and Ecarin (E), that activate prothrombin but differ in their phospholipid requirements. By testing this new assay we have evaluated 36 patient plasmas containing LA according to standard tests (activated partial thromboplastin time, dilute Russell viper venom time and platelet neutralization procedure) and our results confirm a high sensitivity for LA of the T/E test.In addition, we observed a greater sensitivity of the tissue thromboplastin inhibition test using a recombinant thromboplastin instead of a human placenta thromboplastin.Our study also showed that the T/E test seems to be a useful assay in confirming the diagnosis of LA in patients with an unexplained prolonged APTT.

Relative Sensitivity of Different Tests in the Detection of Low Titer Lupus Anticoagulants

1988 ◽  
Vol 60 (02) ◽  
pp. 217-219 ◽  
Author(s):  
B Lesperance ◽  
M David ◽  
J Rauch ◽  
C Infante-Rivard ◽  
G E Rivard
Keyword(s):  
Relative Sensitivity ◽  
Fetal Outcome ◽  
Anticardiolipin Antibodies ◽  
Lupus Anticoagulants ◽  
Normal Plasma ◽  
Coagulation Tests ◽  
High Dilutions ◽  
Tissue Thromboplastin ◽  
High Concentrations ◽  
Kaolin Clotting Time

SummaryLupus anticoagulants (LA) and anticardiolipin antibodies have been strongly associated with recurrent abortion and fetal death. Because steroids have been reported to improve the fetal outcome of LA associated pregnancies, presumably by decreasing the levels of LA, it becomes desirable to have a simple and reliable test to monitor the levels of the putative antibody. To this effect, we assessed the capacity of the following coagulation tests to detect the presence of LA in serial dilutions of patient plasma with pooled normal plasma: kaolin clotting time (KCT), tissue thromboplastin inhibition test (TTIT), dilute Russell Viper venom time (DRVVT) and activated partial thromboplastin time with standard and high concentrations of phospholipids (SC and HCAPTT). All samples were also evaluated for the presence of anticardiolipin antibodies with an ELISA. The KCT was able to detect LA at a much greater dilution in normal plasma than any of the other clotting assays. The ELISA was comparable to KCT in its ability to detect high dilutions of LA.

Scientific and Standardization Committee Communications Comparison of Test Methods for the Lupus Anticoagulant: International Survey on Lupus Anticoagulants-I (ISLA-1)

1990 ◽  
Vol 64 (03) ◽  
pp. 478-484 ◽  
Author(s):  
Thomas Exner ◽  
Douglas A Triplett ◽  
David A Taberner ◽  
Margaret A Howard ◽  
E Nigel Harris
Keyword(s):  
Lupus Anticoagulant ◽  
Test Methods ◽  
Positive Sample ◽  
Direct Proportion ◽  
Clotting Time ◽  
Preliminary Screening ◽  
Activated Platelets ◽  
Tissue Thromboplastin ◽  
Kaolin Clotting Time ◽  
Induced Defects

SummarySix lyophilized plasma samples were sent to 20 “expert” laboratories for assessment of lupus anticoagulant (LA). Four samples contained pooled LA of graded potency mixed with aged normal plasma. One contained LA plus cephalin phospholipid and one contained a nonspecific venom anticoagulant. Sixteen methods were used overall with some participants using up to 8 methods. Results were scored in regard to the known potencies of LA in the samples and other known induced defects.Activated partial thromboplastin time (APTT) tests used by most participants for preliminary screening were relatively sensitive, but non-specific. Platelet or phospholipid neutralization procedures (PNP) appeared to be sensitive and specific but showed a non-linear response to increased LA content. Kaolin clotting time (KCT) tests showed the most sensitive response to increased LA content but the weaker LA were not scored as abnormal by most laboratories as the samples may have contained platelet fragments. Other commonly used tests such as the tissue thromboplastin inhibition (TTI) test and the dilute Russell’s viper venom test (DRVVT) were carried out somewhat inconsistently. The variability in performance of tests in different laboratories indicates that standardization of methodology is urgently required.Generally it seemed that most clotting tests were “bypassed” by the addition of phospholipid to a known LA-positive sample in apparently direct proportion to their sensitivity. Sample preparation, especially prevention of contamination with activated platelets is a vital preliminary part in the assay of LA.

Cerebral Amyloid Angiopathy in Combination with Paroxysmal Atrial Fibrillation

2020 ◽  
Vol 25 (4) ◽  
pp. 31-37
Author(s):  
A. A. Kornilova ◽  
O. V. Lagoda ◽  
M. M. Tanashyan
Keyword(s):  
Atrial Fibrillation ◽  
Cerebral Amyloid Angiopathy ◽  
Paroxysmal Atrial Fibrillation ◽  
Past History ◽  
Laboratory Data ◽  
Clinical Manifestations ◽  
Cerebral Haemorrhage ◽  
Exclusion Criterion ◽  
Amyloid Angiopathy ◽  
Cerebral Amyloid

The present article addresses the definition of cerebral amyloid angiopathy (CAA) and its symptoms based on the analysis of the medical case; the issues of diagnosis and treatment of this pathology are discussed. The Boston criteria, which became the basis for diagnosis, study of clinical manifestations and progression of CAA and approaches to its therapy, are presented. Methods and modes of neuroimaging, including magnetic resonance imaging (MRI), which verify micro cerebral haemorrhage, are described. At the same time, the role and significance of cardiac arrhythmias in the genesis of ischemic stroke are discussed, and scales for assessing the risk of its occurrence are presented. The observation of the neurological, somatic, neuroimaging, neuropsychological status of a 62-year-old patient confirms quite rare combination of probable CAA, paroxysmal atrial fibrillation and repeated hemorrhagic functional apoplexy (FA). The relevance of the case described, is a complex clinical dilemma based on mutually exclusive recommendations for the pharmacological correction of such conditions. It is emphasized that in many multicenter clinical studies on the effectiveness of antithrombotic medication (antiaggregants, anticoagulants) in the treatment and prevention of ischaemic functional apoplexy , an important exclusion criterion is a hemorrhagic stroke in past history (including the multiple changes in haemostasis indicators). Taking into account the obtained clinical and laboratory data in the dynamics, the tactics of treating the described patient were determined. The results of studies related to the treatment of comorbid pathology that should become the subject of the development of a personalized algorithm for managing patients in each specific case, are discussed.

Thrombotic Events in Homozygotes with a Proven or Highly Probable Arg304Gln Factor VII Mutation (FVII Padua)1): Only Limited Replacement Therapy is Needed in Case of Surgery

2019 ◽  
Vol 19 (3) ◽  
pp. 233-238
Author(s):  
Antonio Girolami ◽  
Elisabetta Cosi ◽  
Silvia Ferrari ◽  
Bruno Girolami ◽  
Maria L. Randi
Keyword(s):  
Replacement Therapy ◽  
Thrombotic Event ◽  
Factor Vii ◽  
Compound Heterozygous ◽  
Activity Levels ◽  
High Prevalence ◽  
Molecular Studies ◽  
Tissue Thromboplastin ◽  
The Difference ◽  
Compound Heterozygotes

Objective: To investigate the prevalence of thrombotic events among patients with proven or highly probable homozygosis for the Arg304Gln (Factor VII Padua) defect or compound heterozygosis containing the Arg304Gln mutation. Methods: Homozygotes and compound heterozygotes proven by molecular studies to have the Arg304Gln mutation were gathered from personal files and from two PubMed searches. In addition, patients with probable homozygosis on the basis of clotting tests (discrepancies among Factor VII activity levels according to the tissue thromboplastin used) were also gathered. Results: 30 proven homozygotes and 17 probable ones were gathered together with 8 compound heterozygotes. In the latter use, the associated mutation was Cys135Arg (twice), Gly180Arg, Arg304Trp, Arg315Trp, His348Gln, Gly365Cys. The prevalence of venous thrombotic events was 16.6, 11.8 and 11.1 percent, respectively for the three groups of patients. Heterozygotes showed no thrombotic event. The difference for proven homozygotes was statistically significant, while for the other groups only a trend was present. Conclusion: proven homozygous or compound heterozygous patients with the Arg304Gln mutation showed a higher than expected incidence of thrombotic events. The same is true for probable cases gathered only on the basis of clotting tests. These patients, because of their frequent lack of bleeding and for their relatively high prevalence of thrombosis should probably receive only limited replacement therapy in case of surgical procedures.

Changes In Prothrombin And Activated Partial Thromboplastin Time During Replacement Therapy With Human Recombinant Growth Hormone In Growth Hormone Deficient Adults

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S102-S103
Author(s):  
Y S Kamel
Keyword(s):  
Growth Hormone ◽  
Replacement Therapy ◽  
Healthy Subjects ◽  
Recombinant Growth Hormone ◽  
Gh Replacement ◽  
Coagulation Tests ◽  
Before And After ◽  
Human Recombinant Growth Hormone ◽  
Male Patients ◽  
One Year

Abstract Introduction/Objective The aim of this study was to investigate the effects of GH administration on basic coagulation parameters: PT, aPTT and fibrinogen concentrations in adult GHD patients before and during one year of GH replacement. Methods Twenty-one adult patients with severe GHD (mean age +/- SE: 38.6 +/- 2.8 years) were included in this hospital based, prospective, interventional study. All patients were treated with rhGH for 12 months (GH dose: 0.4 mg/day for male and 0.6 mg/day for female patients). IGF-1 concentrations were determined using RIA-INEP kits. Basic coagulation tests, i.e. aPTT and fibrinogen concentrations, were measured before and after 3, 6 and 12 months of treatment with rhGH. Control values were obtained from fourteen “healthy” subjects matched by age, sex and body mass index (BMI). Results At baseline, we observed no significant differences in PT, aPTT and fibrinogen values between GHD and healthy subjects. IGF-1 concentrations increased significantly within 3 months of GH therapy (8.2 +/- 1.5 vs. 24.2 +/- 2.9 nmol/l, p &lt;0.05) and remained stable thereafter. A significant increase in PT values, which was more pronounced in female subjects, was noted after 6 and 12 months of treatment with GH. aPTT values increased significantly after 12 months of treatment only in male patients (28.8 +/- 4.6 vs. 39.7 +/- 2.1 s.; p &lt;0.05). No significant changes in fibrinogen concentrations were found during the study. Conclusion Twelve months of GH replacement therapy led to a significant increase in PT and aPTT values in adult GHD patients, while fibrinogen concentrations did not change. Changes in PT were more pronounced in female GHD patients, while an increase in aPTT values was observed only in male patients with GHD. The clinical significance of these changes needs further evaluation.

A GENERAL MECHANISM FOR LUPUS ANTICOAGULANTS

1987 ◽  
Author(s):  
V Pengo ◽  
M J Heine ◽  
P Thiagarajan ◽  
s s Shapiro
Keyword(s):  
Lupus Anticoagulant ◽  
Anticoagulant Activity ◽  
Protein A ◽  
Coagulation Factors ◽  
Phosphatidyl Serine ◽  
General Mechanism ◽  
Factor X ◽  
Lupus Anticoagulants ◽  
Calcium Dependent ◽  
Anionic Phospholipids

Although- a number of observations have implied that lupus anticoagulants have immunologic specificity towards anionic. phospholipids, thereby prolonging phospholipid-dependent coagulation tests, this assumption has been directly demonstrated in only one patient with a monoclonal IgM paraprotein. We have tested the generality of this hypothesis directly by isolating five IgG lupus anticoagulants from patients with lupus-like syndromes and/or thrombosis. IgG lupus anticoagulant fractions were isolated free of other plasma proteins and free of contaminating phospholipid by adsorption to and elution from cardiolipin-cholesterol-dicetylphosphate liposomes , followed by chromatography on protein A-Sepharose. Cardiolipin liposomes, but not phosphatidylcholine liposomes, were capable of removing all, or nearly all, lupus anticoagulant activity from patient plasma. Anticardiolipin and lupus anticoagulant activity were both present in acidic fractions on isoelectric focusing. F(ab’)2 fragments retained lupus anti coagulant activity and bound to cardiolipin in an ELISA assay. The affinity-purified IgG preparations reacted with cardiolipin, phosphatidyl serine , phosphatidylinositol and phosphatidic acid, but not with phosphatidylcholine or phosphatidyl ethanol amine, and inhibited calcium-dependent binding of prothrombin and of factor X to phosphatidy1serine-coated surfaces. These data demonstrate a general mechanism for the action of lupus anticoagulants: antibodies that have immunologic specificity towards anionic phospholipids, thereby blocking the calcium-mediated binding of vitamin K-dependent coagulation factors to coagulation-active phospholipid surfaces.

Disseminated Intravascular Coagulopathy

1979 ◽  
Vol 1 (2) ◽  
pp. 37-45
Author(s):  
James J. Corrigan
Keyword(s):  
Septic Shock ◽  
Liver Disease ◽  
Laboratory Data ◽  
Purpura Fulminans ◽  
Coagulation Factors ◽  
Screening Tests ◽  
Complete Analysis ◽  
Thrombin Time ◽  
Severe Respiratory Distress ◽  
Severe Respiratory Distress Syndrome

Disseminated intravascular coagulation is most common in children with bacterial septic shock, infants with severe respiratory distress syndrome, in giant hemangiomas, and in purpura fulminans. The diagnosis is suspected when purpuric bleeding and/or thrombosis occurs in those clinical settings known to have DIC associated with them. The coagulopathy is also suspected when the patient has thrombocytopenia and prolonged clotting times in coagulation screening tests (prothrombin time and partial thromboplastin time). Confirmation of the diagnosis requires further laboratory data. Although it would be ideal to have a complete analysis of all the coagulation factors, this is generally not universally available nor always necessary in all cases. In the absence of liver disease, the minimal criteria for diagnosis is the combination of thrombocytopenia, positive fibrinolytic split products, and hypofibrinogenemia (or prolonged thrombin time). In the presence of liver disease, reduced factor VIII and/or the presence of circulating soluble fibrin complexes would be needed to diagnose DIC. Once the diagnosis is established and the underlying illness identified and treated, the therapy of the DIC is generally supportive. Anticoagulation (heparin) management is rarely needed except in cases of purpura fulminans, where it can be lifesaving, and selected cases of giant hemangioma or malignancy. In cases of septic shock and in severe RDS, successful management of the shock and the respiratory failure will cause the DIC to disappear without the use of anticoagulation.

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