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.

Acquired Factor VIII Inhibitor: A Single Institution Experience With 62 Patients

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 3610-3610
Author(s):  
Ewa M. Wysokinska ◽  
Ramila Mehta ◽  
Diane Grill ◽  
Rajiv K. Pruthi
Keyword(s):  
Factor Viii ◽  
Hemophilia A ◽  
Conflicts Of Interest ◽  
Post Partum ◽  
Rare Condition ◽  
Factor Viii Inhibitor ◽  
Remission Rates ◽  
Autoimmune Conditions ◽  
Viii Inhibitor ◽  
Acquired Factor Viii Inhibitor

Abstract Background Acquired Factor VIII inhibitor or autoimmune hemophilia A (AHA), has an estimated incidence of up to 1.5 cases per million/year and may result in severe hemorrhagic complications and death. Approximately 50% of cases have an underlying condition such as malignancies, autoimmune disorders and post-partum state. AHA should be suspected in any patient presenting with unexplained bleeding and an inhibited aPTT. Management consists of maintaining hemostasis and elimination of the inhibitor, however management is not standardized. We present 62 cases of AHA managed at Mayo Clinic Rochester, over the course of 36 years. We also analyzed whether aPTT at presentation correlated with the strength of inhibition measured by Bethesda Titer. Methods After IRB approval, medical records of patients with AHA were reviewed and all clinical data collected. Cumulative incidence of death was estimated by Kaplan-Meier analysis. Spearman correlation was used to calculate relation of APTT to Bethesda titer. Results Between 1976 and 2012, we identified 62 patients (male: 35), with a median age at diagnosis of AHA 69 years (mean 64, range 20-86). Clinical presentation consisted of extensive ecchymoses (n=40, 64%) in majority of cases. 29/62 (47%) patients had at least 1 identifiable predisposing condition with 12/62 (19%) patients with an underlying malignancy and 16/62 (26%) with underlying autoimmune conditions. Median Bethesda titer was 29 (range 1 to 1178). Bethesda titer was not related to the number or duration of hospitalizations. Most (69%) patients had at least one hospitalization and 12 (19%) had more than one hospitalization for bleeding complication. Inpatient therapy for bleeding consisted most commonly of FEIBA in 21 pts (34%) and rFVIIa in 6 pts (10%). Prednisone was the most common immunosuppressant used in 54 (87%) patients while Rituximab was used in 11 (18%) patients. Of 32 patients with available follow up labs most (69%) achieved remission. There was no difference in remission rates between patients treated or not treated with Rituximab (p=0.1735). Conclusion Acquired Hemophilia A is a rare condition with very heterogenous presentation. It affects mostly older male patients who present with ecchymoses and elevated APTT. The degree of APTT prolongation at the time of diagnosis does not correlate with the strength of the Bethesda titer and should not guide choice of therapy in a patient presenting with an acute bleed. Rituximab use in the 11 patients treated at Mayo did not seem to influence remission rates or survival. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Acquired Factor VIII Inhibitor in a Non-Hemophilic Patient with Chronic Hepatitis C Viral Infection.

1999 ◽  
Vol 38 (3) ◽  
pp. 283-286 ◽  
Author(s):  
Kazuro SUGISHITA ◽  
Hiroyuki NAGASE ◽  
Toshiyuki TAKAHASHI ◽  
Katsu TAKENAKA ◽  
Yasushi SHIRATORI ◽  
...  
Keyword(s):  
Chronic Hepatitis ◽  
Hepatitis C ◽  
Viral Infection ◽  
Factor Viii ◽  
Chronic Hepatitis C ◽  
Factor Viii Inhibitor ◽  
Hemophilic Patient ◽  
Viii Inhibitor ◽  
Acquired Factor Viii Inhibitor

An Acquired Factor VIII Inhibitor Responsive to High-Dose Gamma Globulin

1987 ◽  
Vol 58 (04) ◽  
pp. 1005-1007 ◽  
Author(s):  
David Green ◽  
Hau C Kwaan
Keyword(s):  
Factor Viii ◽  
Gamma Globulin ◽  
Clotting Factor ◽  
Uneventful Recovery ◽  
High Dose ◽  
Factor Viii Inhibitor ◽  
Minimal Trauma ◽  
Viii Inhibitor ◽  
Acquired Factor Viii Inhibitor ◽  
Clotting Factor Concentrates

SummaryA 55-year-old previously well woman noted easy bruising and developed a swollen, painful leg after minimal trauma. A compartment syndrome was diagnosed, and medial and lateral fasciotomies were performed with evacuation of a massive hematoma. However, blood rapidly reaccumulated in the wound. The VILI : C level was 2%, and 4 Bethesda units of factor VIII inhibitor were detected. After initial treatment with clotting factor concentrates and corticosteroids failed to control bleeding or reduce inhibitor titers, gamma globulin, 25 g daily for 5 days, was administered. The inhibitor became undetectable, VIII :C levels rose, and bleeding stopped. However, 5 days later VIII :C levels were again low and bleeding recurred. A second course of gamma globulin, 50 g daily for 2 days, was accompanied by a prompt increase in VIII :C, and uneventful recovery. In conclusion, in this patient with an autoantibody to Vili : C, a response to gamma globulin was observed on two occasions, and the second response came when steroids were being tapered and the patient was on no other medication.

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.

Rituximab in the Treatment of Factor VIII Inhibitor.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4064-4064 ◽  
Author(s):  
Mercedes Maruscak ◽  
Barry Cohan ◽  
Linda Aghakhanian ◽  
Kalust Ucar
Keyword(s):  
Hepatitis C ◽  
Factor Viii ◽  
Life Support ◽  
Low Grade ◽  
Factor Viii Inhibitor ◽  
Hepatitis C Infection ◽  
Fda Approval ◽  
Viii Inhibitor ◽  
Liver Biopsies ◽  
Acquired Factor Viii Inhibitor

Abstract Rituximab is a monoclonal antibody directed against the CD20 antigen found on both malignant and normal B lymphocytes. It was given FDA approval in 1997 for relapsed or refractory, low grade or follicular, CD20+ non-Hodgkin’s lymphoma. We report the use of selective B-cell depletion with Rituximab on a fifty-nine year old male with acquired Factor VIII inhibitor who failed to respond to convential therapy. The patient presented with compartment syndrome of the left calf due to spontaneous severe hematoma, and was subsequently hospitalized. The patient was found to have a PTT of 86, Factor VIII <3%, and Factor VIII inhibitor 108 b.u. The patient had completed forty-eight weeks of interferon and ribavirin for Hepatitis C one month prior to admission. The PTT was normal nineteen months prior when liver biopsies were obtained. The patient was subsequently diagnosed with acquired Factor VIII deficiency, thought to be secondary to either the Hepatitis C infection or the interferon therapy. He improved with Factor VIII concentrate therapy, but did not spontaneously recover from the inhibitor. He had minimal response to IVIG. Rituxin was subsequently started (intravenously at 375mg/m2) once a week for eight consecutive weeks, and repeated every six months. After four cycles, a consistent response was documented by increased Factor VIII activity (as high as 62%), and decreased Factor VIII inhibitor (stable at <0.5%). The treatment was well tolerated, and no infusion related side effects were observed. The patient did well with no bleeding on maintenance therapy for approximately twenty-one months. Unfortunately, two weeks after completion of the fourth cycle the patient suffered a ruptured AAA, and upon surgical intervention became unresponsive and a right cerebral infarct was identified. The patient’s family subsequently discontinued life support and the patient died. This experience suggests that further investigation of Rituximab therapy in this defined population might benefit similar patients.

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.

Sign in / Sign up

Export Citation Format

Share Document