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

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.

Immunological Analysis of Acquired Factor VIII Inhibitor in a Case with Immunologic Disorder

1991 ◽  
Vol 85 (3) ◽  
pp. 153-155 ◽  
Author(s):  
Takashi Okamura ◽  
Yasuo Yamauchi ◽  
Takahiro Fukuda ◽  
Kazuhisa Suehiro ◽  
Masahiro Murakawa ◽  
...  
Keyword(s):  
Factor Viii ◽  
Factor Viii Inhibitor ◽  
Immunological Analysis ◽  
Viii Inhibitor ◽  
Acquired Factor Viii Inhibitor

Acquired factor VIII inhibitor in a non-haemophilic boy

Haemophilia ◽  
2003 ◽  
Vol 9 (5) ◽  
pp. 642-645 ◽  
Author(s):  
A. Klukowska ◽  
P. Łaguna ◽  
A. Obitko-Płudowska ◽  
K. Niedzielska ◽  
I. Malinowska ◽  
...  
Keyword(s):  
Factor Viii ◽  
Factor Viii Inhibitor ◽  
Viii Inhibitor ◽  
Acquired Factor Viii Inhibitor

Role of Plasmapheresis In Management of Acquired Factor VIII Inhibitor

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4656-4656
Author(s):  
Ratesh Khillan ◽  
Rabia Latif ◽  
Gurinder Sidhu ◽  
Elizabeth Gloster ◽  
Albert S. Braverman ◽  
...  
Keyword(s):  
Factor Viii ◽  
Ct Scan ◽  
Case Reports ◽  
High Dose ◽  
Factor Viii Inhibitor ◽  
Inhibitor Level ◽  
Viii Inhibitor ◽  
Acquired Factor Viii Inhibitor ◽  
Dose Factor

Abstract Abstract 4656 A 91-year-old woman with past medical history of hypertension presented with hematuria. There were no ecchymosis, Petechiae or other obvious active bleeding. Her hemoglobin was 11.4 g/dl on presentation hematuria got worse and her hemoglobin drops to 7.6 g/dl over next 48 hours and she was hemodynamically unstable. She was transferred to the Medical Intensive Care Unit for resuscitation with IV fluids and PRBCs. Coagulation tests revealed a prolongation of activated partial thromboplastin time of more than 100 seconds (control 33 seconds) which could not be corrected with mixing normal plasma. Diagnosis of acquired factor VIII inhibitor was considered and recombinant activated factor VII (rFVIIa) was initiated. The factor VIII activity level was reduced to less than 1%. Bethesda assay demonstrated the presence of a factor VIII inhibitor at 103.8 Bethesda units per ml (BU/ml), other coagulation studies were with in normal range. CT scan of her abdomen showed retroperitoneal hematoma. rFVIIa was started at 50 units/kg body weight every 3 hours and subsequently increased to 200 units/kg. She was simultaneously started on steroids. Her hematuria did not improve in spite of high dose rFVIIa. On day 4 rFVIIa was tapered and switched to 50 units/kg FEIBA (Factor eight inhibitor bypass agent). She also received Rituximab 375 mg/m2. We continued FEIBA until day 7 but her hematuria did not improve, she required more than 10 units of Packed Red Blood Cells PRBCs during this period. On day 7 we decided to start plasmapheresis as there were some case reports of using plasmapheresis with or without immunoadsorption columns (which are currently not available in US). We started plasmapheresis and gave her 2 doses of IVIG (Immunoglobulin). Her pre and post plasmapheresis inhibitor levels were 104 BU/ml and 54 BU/ml respectively. Her urine turned pink and her Prbc demand decreased. A second plasmapheresis was done 2 days later showed significant decrease of inhibitor level from 80 BU/ml to 14.5 BU/ml. Her hematuria resolved by next day. We continued her on FEIBA for three more days she did not have hematuria and she did not require any PRBCs. CT scan of abdomen showed decrease in size of retroperitoneal hematoma. Cyclophosphamide 1000 mg was given for induction of immune tolerance followed by high dose factor VIII (100 IU/KG) as per Bonn protocol. Her factor VIII levels and factor VIII inhibitor levels were checked every day before and after Factor VIII infusion. Her inhibitor level is ranging between 14–16 BU/ml she is not bleeding any more and her abdominal hematoma is resolved. Her pre and post transfusion factor VIII levels ranges between 30–40% and 120–140%. respectively. Patient is still getting factor VIII everyday. Role of plasmapheresis is not very well defined in acquired Factor VIII inhibitor patients. Acquired hemophilia is a rare autoimmune disorder in which the patient develop an autoantibody directed against coagulation factor VIII leading to a clinically bleeding diathesis. There are few case reports in literature showing efficacy of Plasmapheresis in this disorder. This is a rare condition and it is very difficult to find large randomized trial to establish a standard of care. Patient mentioned above did not respond to rFVIIa or FEIBA. In our observation plasmapheresis with IVIG proved to be an effective method of rapidly reducing the inhibitor level. In case of life threatening bleeding we need to reduce the inhibitor level quickly. We also observed that once inhibitor level was low bleeding stopped. Immune induction therapy with cyclophosphamide followed by high dose factor VIII was successful in maintaining low inhibitor level. Disclosures: Kessler: Grifols S.A.: Research Funding.

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.

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