How we treat a hemophilia A patient with a factor VIII inhibitor

Blood ◽  
2009 ◽  
Vol 113 (1) ◽  
pp. 11-17 ◽  
Author(s):  
Christine L. Kempton ◽  
Gilbert C. White
Keyword(s):  
Factor Viii ◽  
Hemophilia A ◽  
Clotting Factor ◽  
Patient Specific ◽  
Factor Viii Inhibitor ◽  
Factor Viii Activity ◽  
Related Factors ◽  
Acute Bleeding ◽  
Viii Inhibitor

Abstract The most significant complication of treatment in patients with hemophilia A is the development of alloantibodies that inhibit factor VIII activity. In the presence of inhibitory antibodies, replacement of the missing clotting factor by infusion of factor VIII becomes less effective. Once replacement therapy is ineffective, acute management of bleeding requires agents that bypass factor VIII activity. Long-term management consists of eradicating the inhibitor through immune tolerance. Despite success in the treatment of acute bleeding and inhibitor eradication, there remains an inability to predict or prevent inhibitor formation. Ideally, prediction and ultimately prevention will come with an improved understanding of how patient-specific and treatment-related factors work together to influence anti–factor VIII antibody production.

scholarly journals Postpartum Acquired Hemophilia: A Rare Cause of Postpartum Hemorrhage

2013 ◽  
Vol 2013 ◽  
pp. 1-2 ◽  
Author(s):  
Srikanth Seethala ◽  
Sumit Gaur ◽  
Elizabeth Enderton ◽  
Javier Corral
Keyword(s):  
Factor Viii ◽  
Hemophilia A ◽  
Factor Vii ◽  
Normal Vaginal Delivery ◽  
Factor Viii Inhibitor ◽  
Activity Levels ◽  
Factor Viii Activity ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Viii Inhibitor

A 36-year-old female started having postpartum vaginal bleeding after normal vaginal delivery. She underwent hysterectomy for persistent bleeding and was referred to our institution. An elevation of PTT and normal PT made us suspect postpartum acquired hemophilia (PAH), and it was confirmed by low factor VIII activity levels and an elevated factor VIII inhibitor. Hemostasis was achieved with recombinant factor VII concentrates and desmopressin, and factor eradication was achieved with cytoxan, methylprednisolone, and plasmapheresis.

scholarly journals Acquired factor VIII inhibitor associated with chronic untreated hepatitis C

2018 ◽  
Vol 6 (23) ◽  
pp. 17-21
Author(s):  
Abdussalam Shredi ◽  
Benjamin W. Elberson ◽  
Saif El Nawaa ◽  
Amr Ismail
Keyword(s):  
Hepatitis C ◽  
Factor Viii ◽  
Hemophilia A ◽  
Autoimmune Disorders ◽  
Hcv Infection ◽  
Clotting Factor ◽  
Prolonged Treatment ◽  
Factor Viii Inhibitor ◽  
Viii Inhibitor ◽  
Acquired Factor Viii Inhibitor

Acquired inhibitors of coagulation are antibodies that either inhibit the activity or increase the clearance of a clotting factor. A hemorrhagic diathesis is a common clinical manifestation in affected patients. Acquired factor VIII inhibitor – or acquired hemophilia A – is a rare disorder and presents similarly to hemophilia A, though patients are less likely to develop hemarthroses. This inhibition is most commonly due to autoantibodies against coagulation factor VIII. These autoantibodies often occur in pregnancy, autoimmune disorders, solid tumors, and lymphoproliferative syndromes. Several drugs, including penicillins, phenytoin, and sulfa drugs, have also been associated with antibodies to factor VIII. Chronic infection with the hepatitis C virus (HCV), in addition to various degrees of liver inflammation and fibrosis, can have extrahepatic manifestations, especially autoimmune disorders. The most common hematological complications of HCV infection are thrombocytopenia, cryoglobulinemia, and anti-cardiolipin antibodies. A few cases of factor VIII inhibitors occurring in HCV patients have been reported, with a higher incidence after prolonged treatment with interferon-α. Here, we present a case of a patient with chronic untreated HCV infection developing acquired factor VIII deficiency.

scholarly journals Idiopathic Acquired Hemophilia A with Undetectable Factor VIII Inhibitor

2014 ◽  
Vol 2014 ◽  
pp. 1-4
Author(s):  
Nicholas B. Abt ◽  
Michael B. Streiff ◽  
Christian B. Gocke ◽  
Thomas S. Kickler ◽  
Sophie M. Lanzkron
Keyword(s):  
Factor Viii ◽  
Hemophilia A ◽  
Term Therapy ◽  
Factor Viii Inhibitor ◽  
Factor Viii Activity ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Fviii Activity ◽  
Viii Inhibitor ◽  
Acquired Factor Viii Inhibitor

Objective. We present the case of a 73-year-old female, with no family or personal history of a bleeding disorder, who had a classic presentation for acquired hemophilia A. Factor VIII activity was low but detectable and a factor VIII inhibitor was undetectable.Methods. The patient’s plasma was comprehensively studied to determine the cause of the acquired coagulopathy. Using the Nijmegen modification of the Bethesda assay, no factor VIII autoantibody was measureable despite varying the incubation time from 1 to 3 hours.Results. The aPTT was prolonged at 46.8 seconds, which did not correct in the 4 : 1 mix but did with 1 : 1 mix. Using a one stage factor VIII activity assay, the FVIII activity was 16% and chromogenic FVIII activity was also 16%. The patient was treated with recombinant FVII and transfusion, significantly reducing bleeding. Long-term therapy was initiated with cyclophosphamide and prednisone with normalization of FVIII activity.Conclusions. Physicians can be presented with the challenging clinical picture of an acquired factor VIII inhibitor without a detectable inhibitor by the Bethesda assay. Standard therapy for an acquired hemophilia A should be considered.

scholarly journals A Clinical and Experimental Study of Acquired Inhibitors to Factor VIII

Blood ◽  
1965 ◽  
Vol 26 (6) ◽  
pp. 805-818 ◽  
Author(s):  
HAROLD R. ROBERTS ◽  
MARGARET B. SCALES ◽  
JOHN T. MADISON ◽  
WILLIAM P. WEBSTER ◽  
GEORGE D. PENICK
Keyword(s):  
Experimental Study ◽  
Factor Viii ◽  
Mode Of Action ◽  
Exchange Transfusion ◽  
Hemophilia A ◽  
Retroperitoneal Hematoma ◽  
Factor Viii Inhibitor ◽  
Viii Inhibitor ◽  
Potent Factor

Abstract Factor VIII inhibitors which developed in four patients with hemophilia A are described. These inhibitors are apparently specific for Factor VIII and are capable of inducing a transient hemophilic state when injected into dogs. The genesis, properties, and mode of action of these inhibitors can be explained on an immunologic basis and it seems most likely that they represent an antibody to Factor VIII. One hemophilia A patient, with retroperitoneal hematoma and a potent Factor VIII inhibitor, was successfully treated by an exchange transfusion followed by administration of purified porcine Factor VIII.

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

Hemophilic Dog Model For Evaluating Hemostatic Efetcacy Of Plasma Protein Fractions

1981 ◽  
Author(s):  
H S Kingdon ◽  
T M Hassell
Keyword(s):  
Factor Viii ◽  
Plasma Protein ◽  
Hemophilia A ◽  
Protein Fractions ◽  
Factor Viii Inhibitor ◽  
Repeat Dose ◽  
The Usa ◽  
Sharp Dissection ◽  
Viii Inhibitor ◽  
Uncontrolled Bleeding

About 15% of patients with hemophilia A develop inhibitors to Factor VIII. Because in many cases the inhibitor renders the patient refractory to treatment with Factor VIII, plasma protein fractions designed to bypass the inhibitor have been developed. In the USA these are referred to genetically as Anti Inhibitor Ccrplex Concentrates (AICCs). Development of AICCs has been hampered by lack of a suitable irodelin which to judge hemostatic efficacy. Similarly, lack pf a model has impeded research on the mechanism of action Of AICCs. Therefore, we chose to evaluate AICCs in dogs with hemophilia A, reasoning that a material capable of bypassing & Factor VIII inhibitor should be effective in Factor VIII deficient recipients with or without inhibitors. Under local anesthesia a standardized gingival biopsy was performed losing a flexible plastic template and a modified scalpel handle holding two #11 Bard-Parker blades. The parallel time is ions were 5 mm long, 2 nm apart, and 1.5 mm deep. The tissue block thus defined was removed by sharp dissection. In normal dogs, bleeding from this wound ceased in 5 ± 2min, the wound was filled with concave clot, and bleeding did not recur. In contrast, hemophilic dogs formed an abnormal (very large) clot, and rebled for several days if untreated. The hematocrit usually dropped by 2-10 percentage points in 24 hr of uncontrolled bleeding. An experimental AICC fraction under development by Cutter Laboratories was evaluated in 5 dogs, and shown to be hemostatically effective. The dose required to achieve hemostasis was 25-75 units/kg; in some dogs a second dose was required 6 hr after the first dose to maintain hemostasis. A single dog with a low titer inhibitor to Factor VIII was successfully treated with 39 u/kg, followed by a repeat dose of 39 u/kg 6 hr after the first dose. We conclude that this AICC preparation brings about nemostasis in Factor VIII deficient individuals with or without inhibitors to Factor VIII.

Hemodialysis in a patient with severe hemophilia A and factor VIII inhibitor

2016 ◽  
Vol 20 (4) ◽  
pp. E11-E13 ◽  
Author(s):  
Natarajan Gopalakrishnan ◽  
Thiruvengadam Usha ◽  
Balasubramaniyan Thopalan ◽  
Jeyachandran Dhanapriya ◽  
Thanigachalam Dineshkumar ◽  
...  
Keyword(s):  
Factor Viii ◽  
Hemophilia A ◽  
Factor Viii Inhibitor ◽  
Severe Hemophilia ◽  
Viii Inhibitor
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