scholarly journals Treatment of bleeding episodes in haemophilia A complicated by a factor VIII inhibitor in patients receiving Emicizumab. Interim guidance from UKHCDO Inhibitor Working Party and Executive Committee

Haemophilia ◽  
2018 ◽  
Vol 24 (3) ◽  
pp. 344-347 ◽  
Author(s):  
P. W. Collins ◽  
R. Liesner ◽  
M. Makris ◽  
K. Talks ◽  
P. Chowdary ◽  
...  
Keyword(s):  
Factor Viii ◽  
Executive Committee ◽  
Working Party ◽  
Factor Viii Inhibitor ◽  
Haemophilia A ◽  
Bleeding Episodes ◽  
Viii Inhibitor

A Higher than Expected Incidence of Factor VIII Inhibitors in Multitransfused Haemophilia A Patients Treated with an Intermediate Purity Pasteurized Factor VIII Concentrate

1993 ◽  
Vol 69 (02) ◽  
pp. 115-118 ◽  
Author(s):  
Kathelijne Peerlinck ◽  
Jef Arnout ◽  
Jean Guy Gilles ◽  
Jean-Marie Saint-Remy ◽  
Jos Vermylen
Keyword(s):  
Factor Viii ◽  
Randomized Trials ◽  
Specific Factor ◽  
Factor Viii Inhibitor ◽  
Haemophilia A ◽  
Viral Inactivation ◽  
Gradual Decline ◽  
Inhibitor Level ◽  
Factor Viii Concentrates ◽  
Viii Inhibitor

SummaryIn May 1990, 218 patients with haemophilia A regularly attending the Leuven Haemophilia Center were randomly assigned to a group receiving either of two newly introduced factor VIII concentrates: factor VIII-P, an intermediate purity pasteurized concentrate, or factor VIII-SD, a high purity concentrate treated with solvent-detergent for viral inactivation.Patients were followed from May 1990 until October 1991. Between August 1991 and October 1991 a clinically important factor VIII inhibitor was detected in five out of the 109 patients receiving factor VIII-P while none of the 109 patients receiving factor VIII-SD developed such antibodies. All patients acquiring an inhibitor had previously been clinically tolerant to transfused factor VIII with 200 to more than 1,000 days of exposure to factor VIII prior to May 1990. Patients with inhibitors were transfused daily with 30 U factor VIII-SD per kg body weight, which was associated with a gradual decline of the inhibitor level. In all patients the antibodies were relatively slow-acting and predominantly directed towards the light chain of factor VIII.This study demonstrates a higher than expected incidence of factor VIII inhibitors associated with the use of a specific factor VIII concentrate in multitransfused haemophilia A patients. It indicates the usefulness of evaluating newly introduced concentrates in prospective, randomized trials.

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.

A Case of Acquired Hemophilia Post Treatment with Recombinant Human Activated Protein C (rh APC).

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4021-4021
Author(s):  
Yelena Patsiornik ◽  
Archana Maini
Keyword(s):  
Factor Viii ◽  
Factor Vii ◽  
Factor Viii Inhibitor ◽  
Factor Xii ◽  
Operating Table ◽  
Apache Score ◽  
Acquired Hemophilia ◽  
Drotrecogin Alfa Activated ◽  
Bleeding Episodes ◽  
Viii Inhibitor

Abstract A 87-year old male without a diagnosis of hemophilia presented to the ER with a large shoulder hematoma, developing after a minor fall. Activated partial thromboplastin time (aPTT) was 79.4 sec, not correcting on mixing studies. Factor assays showed a factor VIII of 11.9 %, factor IX of 74.7 %, factor XI of 82.2 % and factor XII of 65.5 %. Anticardiolipin IgG, IgM antibodies and lupus anticoagulant were negative. Factor VIII inhibitor was found and initially measured at 14 Bethesda Unit (BU). A diagnosis of Acquired Hemophilia was made and the patient was treated with recombinant factor VII (rVIIa) and factor VII concentrates, FFP, PRBC transfusions and steroids. The patient had 4 bleeding episodes during hospitalization. First episode was not treated because of the lack of correct diagnosis. However, rVIIa was administered for all bleeding episodes and prior to surgical procedures like tracheostomy. Interestingly, despite immunosupression with steroids, the inhibitor titer did not decrease, rather it displayed a greater variation in the BU values ranging from 14 to 67. The patient bled unpredictably at different titers of inhibitor, without any concordance between the bleeding or the aPTT or the BU value. Finally,he succumbed to uncontrolled bleeding from the tracheostomy site and expired on operating table on day 30 of his fateful admission. This is a unique case of Acquired Hemophilia as 8 months ago this patient had received rhAPC, also known as Drotrecogin Alfa Activated (DAA) for severe sepsis with APACHE score of 25. DAA has a newly established therapeutic role in the inhibitor of factors Va and VIIIa, limiting the thrombotic effect and thus the mortality of sepsis. However, cost-effectiveness and safety of DAA remain somewhat controversial. Despite its recent entry into the medical armamentarium of our fight against sepsis, there exist several references in the published literature about the need of a confirmatory prospective trial about its role even in patients with a high APACHE score. Although neutralizing antibody against APC have not been detected in any patient and there are no published reports about DAA induced antibodies to factor VIII, it remains a viable hypothesis nonetheless. Unknown mechanisms such as exposure of new epitopes of factor VIII degraded by rhAPC could precipitate factor VIII antibodies. Further, the unpredictable titer of factor VIII inhibitor may be secondary to the unique mode of antibody formation in this instance. Lack of Correlation between Factor VIII inhibitor Titers and Clinical Sequelae Dates PTT PT Hb/Ht F VIII inhibitor(BU) Bleeding Episodes 2/01/05 151.4 32 7.1/22.4 On admission: soft tissue hematoma 2/02/05 79.4 14.8 6.2/18.2 14 2/04/05 84.6 16.2 9.3/27.7 29.8 2/05/05 75.2 10 11/32.9 2/06/05 74.8 16.1 9.8/29.7 2/07/05 90.4 17.5 9.2/26.5 67 2/08/05 162.0 17.0 8.8/26.7 GI bleeding, hematuria 2/10/05 97.4 14.1 7.5/22 2/13/05 119 14.6 7.4/22.7 3/01/05 113.8 14.1 9.4/29.8 35 3/02/05 9.2/28.8 Hematuria, rectal bleeding 3/03/05 86.2 12 7/21.2 Bleeding from tracheostomy site

A case report of a premature infant with haemophilia A and factor VIII inhibitor

Haemophilia ◽  
2011 ◽  
Vol 17 (4) ◽  
pp. 711-712 ◽  
Author(s):  
P. CARTLEDGE ◽  
K. DEAKIN ◽  
L. McKECKNIE ◽  
M. RICHARDS
Keyword(s):  
Case Report ◽  
Factor Viii ◽  
Premature Infant ◽  
Factor Viii Inhibitor ◽  
Haemophilia A ◽  
Viii Inhibitor
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