New Method for Quantifying Lupus Anticoagulant

1996 ◽  
Vol 2 (4) ◽  
pp. 237-240
Author(s):  
Roy Speck
Keyword(s):  
Factor Viii ◽  
Confidence Level ◽  
Lupus Anticoagulant ◽  
Heparin Therapy ◽  
Factor Ix ◽  
New Method ◽  
Factor Viii Inhibitor ◽  
Single Factor ◽  
Viii Inhibitor

A method is presented using a new reagent containing propylgallate for the quantitative determina tion of lupus anticoagulant. The amount of an optimized phospholipid standard required by the clotting reaction was found to be 32-50 μg/ml at a 95% confidence level, with a mean of 41 μg/ml. This method eliminates the ef fect of heparin therapy, coumadin therapy, factor-VIII inhibitor, factor-IX inhibitor, and single-factor deficien cies that afflict presently used lupus anticoagulant screen ing and confirmatory procedures. Using this method, it should be possible to detect lupus anticoagulant in pa tients at a much lower level and follow the effect of ther apy on lupus anticoagulant.

In Vitro Thrombogenicity Tests of Factor IX Concentrates

1979 ◽  
Vol 42 (05) ◽  
pp. 1355-1367 ◽  
Author(s):  
C V Prowse ◽  
A Chirnside ◽  
R A Elton
Keyword(s):  
Factor Viii ◽  
Partial Thromboplastin Time ◽  
Generation Time ◽  
Activated Partial Thromboplastin Time ◽  
Factor Ix ◽  
In Vitro Tests ◽  
Factor Viii Inhibitor ◽  
Factor Ixa ◽  
Viii Inhibitor

SummaryVarious factor IX concentrates have been examined in a number of in vitro tests of thrombogenicity. The results suggest that some tests are superfluous as in concentrates with activity in any of these tests activation is revealed by a combination of the non-activated partial thromboplastin time, the thrombin (or Xa) generation time and factor VIII inhibitor bypassing activity tests. Assay of individual coagulant enzymes revealed that most concentrates contained more factor IXa than Xa. However only a small number of concentrates, chiefly those that had been purposefully activated, contained appreciable amounts of either enzyme.

Clinical Profile of Haemophilia in a Tertiary Care Hospital in Pune, Maharashtra, India

2021 ◽  
Vol 8 (15) ◽  
pp. 968-971
Author(s):  
Sadiq Yunus Mulla ◽  
Sachin Sitaram Pandit ◽  
Sachin Kisan Shivnitwar
Keyword(s):  
Factor Viii ◽  
Tertiary Care Hospital ◽  
Tertiary Care ◽  
Factor Ix ◽  
Clinical Profile ◽  
Factor Viii Inhibitor ◽  
Haemophilia A ◽  
Care Hospital ◽  
Factor Viii Activity ◽  
Viii Inhibitor

BACKGROUND Haemophilia’s are X-linked hereditary blood clotting disorders due to deficiency of factor VIII (haemophilia A) or factor IX (haemophilia B) & also has identical clinical manifestations, screening tests abnormalities and sex-linked genetic transmission. Haemophilia’s result from defects in the factor VIII / IX gene that lead to decreased amount of factor VIII / IX protein, the presence of a functionally abnormal protein, or combination of both. Haemophilia A is a classic example of an X-linked recessive trait. The severity of their bleeding depends on their factor VIII activity level; and, rarely, a woman can have very low factor VIII activity, and present with symptoms of moderate or even severe haemophilia. We wanted to study the clinical profile of patients of haemophilia admitted in a tertiary care hospital. METHODS This is a cross-sectional study enrolling 60 known cases of haemophilia A & B admitted in wards & ICU / attending OPD of a tertiary care hospital. History was obtained in detail & thorough clinical examination was carried out. Precipitating factors for bleeding (spontaneous / minor trauma / major trauma / surgical operation / dental procedure / others), family h / o bleeding were studied in detail. RESULTS Of the total 60 cases of haemophilia, majority (49) of cases were of haemophilia A and 11 cases were of haemophilia B. In the study, majority (28.33 %) of cases belonged to 12 - 20 years age group and the most common presentation was haemarthrosis (61.67 %). 6 patients had factor VIII inhibitor antibodies and all of them were of haemophilia A. CONCLUSIONS Haemarthrosis is the most common clinical presentation of haemophilia and most common cause for haemarthrosis is spontaneous bleeding. Most common joint involved in bleeding was knee joint (target joint). Presence of factor VIII inhibitor antibodies specially in haemophilia A patients is not uncommon. KEYWORDS Haemophilia, Factor VIII, Factor IX

Use of an Activated Prothrombin Complex Concentrate (Auto Factor IX-Hyland) in the Control of Bleeding of Haemophilic (A) Patients with Inhibitor of Factor VIII

1979 ◽  
Author(s):  
E.J. Watson-Williams ◽  
C.F. Abildgaard ◽  
E. A. Turner
Keyword(s):  
Soft Tissue ◽  
Factor Viii ◽  
Prothrombin Complex Concentrate ◽  
Factor Ix ◽  
Factor Viii Inhibitor ◽  
Activated Prothrombin Complex Concentrate ◽  
Prolonged Aptt ◽  
Bleeding Episodes ◽  
Viii Inhibitor ◽  
Flow Study

One of us (C.F.A.) has previously reported the successful use of one of the commercially available prothrombin complex concentrates for the control of bleeding episodes of haemophiltc patients with factor VIII inhibitors. Subsequent batches of these concentrates have not proved consistently effective even in doses of 150 factor IX units/kg every 24 hours. Recently an investigational preparation, Auto Factor IX, has been made available to us. This has a stated factor VIII correctional unit assay for each batch, (based on the ability to correct the prolonged APTT of plasma containing an inhibitor of factor VIII). We used 60-120 units/kg as an IV dose every 12 or 24 hours in the treatment of 24 bleeding episodes in 8 patients with factor VIII Inhibitor. The bleeding episodes were haemarthrosis (12) soft-tissue (6) intralingual (2) lacerations (2) retroperitoneal (1) and epidural (1). Rapid easing of pain and reduction of swelling was noted in all joints and soft tissue bleeds. In the retroperitoneal bleed cessation of bleeding was demonstrated by Technetium 99 Sulfur-colloid flow study, in the patient with epidural bleeding the hematoma was shown to reduce by serial CAT scans. Response was as good as we have come to expect from similar levels of factor VIII concentrate given to patients without an inhibitor. In 23 of the 24 episodes there was a marked reduction of APTT 10 minutes after the completion of the infusion.

Acquired factor VIII inhibitor and positive lupus anticoagulant testing on silica clotting time

2014 ◽  
Vol 37 (1) ◽  
pp. 139-141
Author(s):  
H.-W. Ip ◽  
C.-C. So ◽  
Y.-Y. R. Leung ◽  
J.-Y. Chan ◽  
P. Sin ◽  
...  
Keyword(s):  
Factor Viii ◽  
Lupus Anticoagulant ◽  
Factor Viii Inhibitor ◽  
Clotting Time ◽  
Viii Inhibitor ◽  
Acquired Factor Viii Inhibitor

scholarly journals Successful management of life-threatening hemorrhage in a patient with synchronous lupus anticoagulant and factor VIII inhibitor

2002 ◽  
Vol 36 (4) ◽  
pp. 853-855 ◽  
Author(s):  
Hans A. Brings ◽  
John K. Waas ◽  
Keith R. McCrae ◽  
Henry R. Baele ◽  
Jerry Goldstone
Keyword(s):  
Factor Viii ◽  
Lupus Anticoagulant ◽  
Factor Viii Inhibitor ◽  
Successful Management ◽  
Life Threatening ◽  
Viii Inhibitor

Fibrinopeptide-A (FPA) Generation by Factor IX Concentrates (F-IX-C) and Feiba (factor VIII inhibitor bypassing activity)

1979 ◽  
Author(s):  
V. Hofmann ◽  
M. Ermanni ◽  
P.M. Straub
Keyword(s):  
Factor Viii ◽  
Factor Ix ◽  
Time Dependent ◽  
Factor Viii Inhibitor ◽  
Fibrinopeptide A ◽  
Coagulant Activity ◽  
At Iii ◽  
Viii Inhibitor

The increase of FPA from 0.4-2.4 to 5.6-80 mg/ml after infusion of F-I-C in 5 hemophiliacs B prompted a study whether thrombin is present or genera ted in F-IX-C and in FEIBA rasp. Purified dialysed fibrinogen served as substrate, FPA-release was measured by RIA and expressed as % of the total FPA in fibrinogen. F-IX-C + fibrinogen produced only traces of FPA. The addition of Ca++ led to a time-dependent increase of FPA-release. 8 batches F-IX-C from 2 sources (Immuno, SwissRedCross) were preincubated with Ca++ for 2 hrs, added to fibrinogen with and without heparin, and FPA measured after 60 s. In absence of heparin 7 F-IX-C produced 5 to 80% FPA, in its presence no FPA was liberated. FEIBA + fibrinogen produced clotting and an 80% FPA-release in 5 min, prevented by prior heparin addition. Preincubation of FEIBA with Ca++ increased FPA-release from 80 to 100%, however, prevention required an ll-fold heparin conc. Hirudin more effectively blocked FPA-release probably due to low AT III in concentrates. Thus, in F-IX-C thrombin is absent or blocked by heparin added by the manufacturer; however, thrombin is rapidly generated in presence of Ca++. The coagulant activity of FEIBA is due to thrombin rather than to a “factor VIII inhibitor bypassing activity”.

scholarly journals Clinical Use of Factor IX Concentrates

1977 ◽  
Author(s):  
D. Ménaché
Keyword(s):  
Beneficial Effect ◽  
Factor Viii ◽  
Radical Change ◽  
Factor Ix ◽  
Severe Bleeding ◽  
Factor Viii Inhibitor ◽  
Anamnestic Response ◽  
Viii Inhibitor ◽  
Factor Ix Deficiency

The clinical efficacy of Factor IX concentrates in the treatment of patients with Factor IX deficiency is well recognized. The availability of such concentrates has brought a radical change in the management of these patients. The basis for treatment is to obtain an effective Factor IX hemostatic level in vivo. In these conditions, concentrates have been used to reduce the incidence of hemorrhagic episodes in patients particularly exposed and more often to control hemorrhagic episodes or to prevent hemorrhage in the post operative period. Although thromboembolic complications have occured in some instances the major indication of Factor IX concentrates still remains replacement therapy in patients with Factor IX deficiency.More recently Factor IX concentrates have been used for the treatment of patients with antibody to Factor VIII. Although the therapeutic principle(s) responsible for the Factor VIII inhibitor bypassing activity has not yet been characterized several beneficial effect of both “activated” and non activated Factor IX concentrates have been observed in such patients experiencing minor or severe bleeding episodes. On the other hand we are aware of some cases without beneficial effect of Factor IX concentrates in patients with Factor VIII inhibitor. The major complication would seem to be an anamnestic response in some patients resulting in either a moderate or a considerable increase of the Factor VIII inhibitor titer when compared to the initial level before Factor IX concentrates therapy. If Factor IX concentrates prove to be efficacious in the treatment of patients with Factor VIII antibody special attention would be required in the manufacturing processing in order to avoid an anamnestic response.

scholarly journals An Unusual Etiology for Elevation of Activated Partial Thromboplastin Time (aPTT) in SLE: Acquired Hemophilia and Lupus Anticoagulant

2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Srikanth Seethala ◽  
Nathaniel P. Collins ◽  
George Comerci
Keyword(s):  
Factor Viii ◽  
Partial Thromboplastin Time ◽  
Lupus Anticoagulant ◽  
Activated Partial Thromboplastin Time ◽  
Factor Viii Inhibitor ◽  
Activity Levels ◽  
First Line ◽  
Acquired Hemophilia ◽  
Viii Inhibitor ◽  
Work Up

A 60-year-old female who has a history significant for diabetes, depression, and rheumatoid arthritis presented with a progressively enlarging hematoma of the left upper extremity. She was found to have an enlarging hematoma and an isolated elevation of activated partial thromboplastin time (aPTT). Lab work-up revealed low factor VIII activity levels and inhibitor titers at 13.38 Bethesda units (BU). Dilute Russell’s viper venom time (dRVVT) revealed a lupus anticoagulant. Hemostasis was achieved with factor VIII inhibitor bypassing activity (FEIBA) and inhibitor eradication with-rituxan after the failure of first-line treatment with cyclophosphamide and prednisone.

scholarly journals Characterization of Factor VIII-Inhibitor By-Passing Activity (FEIBA)

1977 ◽  
Author(s):  
K. Hess ◽  
N. Shih ◽  
G. Tishkoff
Keyword(s):  
Factor Viii ◽  
Factor Xa ◽  
Factor Ix ◽  
Coagulation Cascade ◽  
Factor Viii Inhibitor ◽  
Factor X ◽  
Clotting Factors ◽  
Human Factor Ix ◽  
Viii Inhibitor

In an attempt to identify the thrombogenic factor in human factor IX concentrates, we have studied the role of trace quantities of activated clotting factors employing an assay that compares the Factor VIII-like activity of IX concentrates with the ability of these products to restore to normal the abnormal activated partial thromboplastin time (APTT) of Factor VIII inhibitor plasma after 1 minute and 40 minute incubation. A coagulant activity (FEIBA) was evident when partially purified Factors X and II were combined in vitro. Factor Xa (4 × 10-4 u) plus Factor II gave negative results. Factor IIa (5.5 × 10-2u), when combined with Factor X, generated FEIBA. Activated clotting factors (Xa, IIa) when tested alone, at comparable levels, were devoid of FEIBA. Our results suggest a mechanism, distinct from activated clotting factors, that can effectively by-pass the Factor VIII defect in the coagulation cascade. The proposed mechanism appears to also by-pass the normal inhibitory properties (i.e., antithrombin III) of human blood.

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