scholarly journals PSY17 Cost-Utility Analysis of Immune Tolerance Induction (ITI) Therapy Versus On-Demand Treatment With Recombinant FVII (rFVIIa) for Hemophilia A With High Titer Inhibitors in Iran

2012 ◽  
Vol 15 (7) ◽  
pp. A680
Author(s):  
A. Imani ◽  
H. Rasekh ◽  
M. Golestani
Keyword(s):  
Immune Tolerance ◽  
Hemophilia A ◽  
High Titer ◽  
Tolerance Induction ◽  
Cost Utility ◽  
Cost Utility Analysis ◽  
Utility Analysis ◽  
Immune Tolerance Induction ◽  
On Demand

Successful Treatment of Inhibitor Formation in Adult Congenital Hemophilia A with Rituximab.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4074-4074 ◽  
Author(s):  
Chirag J. Amin ◽  
Alice D. Ma
Keyword(s):  
Factor Viii ◽  
Knee Replacement ◽  
Immune Tolerance ◽  
Bolus Dose ◽  
Hemophilia A ◽  
High Titer ◽  
Tolerance Induction ◽  
Recombinant Factor Viii ◽  
Promising Alternative ◽  
Immune Tolerance Induction

Abstract Inhibitor development in congenital hemophiliacs can be clinically catastrophic. Immune tolerance induction therapy has previously been the standard of care in eradicating inhibitors; however due to a multitude of factors, this may not be applicable in certain patients. The role of Rituximab is receiving more attention in this subset of patients. In this abstract, we report that treatment with Rituximab led to successful eradication of high-titer inhibitors in 3 patients with mild to moderate hemophilia A who developed inhibitors after receiving intensive treatment with recombinant Factor VIII (FVIII). Patient Characteristics: Three patients, aged 50–70, with baseline FVIII levels of 2–9%, developed inhibitors after recombinant Factor VIII infusion. Patient A was treated with continuous infusion FVIII for a post-surgical hemarthrosis for approximately 7 days. Patient B received bolus dose FVIII for a GI bleed for at least 10 days, and Patient C received bolus dose FVIII for knee replacement for 10 days. Factor VIII inhibitors were detected in these patients after one month. None of these patients had been treated with immune tolerance previously or had known inhibitors. Each patient received Rituximab 375mg/m2 every week for 4 weeks total. During and after treatment, FVIII levels and Bethesda inhibitor titers (BU) were monitored. Results: All three patients had eradication of their inhibitors (Figure 1) and return of their FVIII levels to baseline by six months post-treatment. Notably, patient C’s inhibitor peak was 117 BUs, 7 months prior to Rituximab treatment. Patient C’s initial response to Rituximab has been previously reported at ASH in abstract form. We now report that 4 years later, this patient has had a recurrence of his inhibitor after monoclonal FVIII for a contralateral knee replacement but with a peak titer of only 2 (Table 1). Inhibitor Trends after Rituximab Treatment Inhibitor Trends after Rituximab Treatment Bethesda Inhibitor Titer (BU) per Month (*) after Receiving Rituximab 0* 1 3 6 36 48 51 NA=Not applicable as data has not matured yet Patient A (BU) 5 0.7 0 0 NA NA NA Patient B (BU) 17 7 2 0 NA NA NA Patient C (BU) 40 4 0 0 0 2 0.5 Conclusion: Inhibitors in patients with mild-moderate hemophilia differ from those with severe FVIII deficiency, behaving more like the autoantibodies seen in patients with spontaneous FVIII inhibitors. In support of this idea, we successfully treated high titer inhibitors which developed in 3 patients with baseline FVIII levels of 2–9%. All three patients had prompt resolution of their inhibitor titers during the course of therapy, with return of their baseline FVIII levels. Historically, patients with mild-moderate hemophilia treated at the Harold R. Roberts Comprehensive Hemophilia Center at the University of North Carolina were treated either with immune tolerance induction or by bypass agents alone, with inhibitor eradication taking months to years (data not shown). While performance of larger prospective trials would be ideal, the small number of patients with this condition limits the ability to perform these trials. Our findings, in combination with other case series from other institutions, reveal a promising alternative for prompt and reliable treatment in mild-moderate hemophiliacs with inhibitors.

scholarly journals What is the role of an extended half-life product in immune tolerance induction in a patient with severe hemophilia A and high-titer inhibitors?

Hematology ◽  
2016 ◽  
Vol 2016 (1) ◽  
pp. 648-649 ◽  
Author(s):  
Maissaa Janbain ◽  
Steven Pipe
Keyword(s):  
Immune Tolerance ◽  
Half Life ◽  
Hemophilia A ◽  
High Titer ◽  
Tolerance Induction ◽  
High Dose ◽  
Severe Hemophilia ◽  
Immune Tolerance Induction ◽  
History Of

Abstract A 10-year-old boy presents with a history of severe hemophilia A and high-titer inhibitor that had failed high-dose immune tolerance induction (ITI) with a recombinant factor VIII (rFVIII) product and a plasma-derived FVIII product. You are asked by his mother whether he should be tried on ITI with an extended half-life product, in particular, consideration of a rFVIIIFc concentrate.

scholarly journals Immunotolerance induction in a pediatric hemophilic patient in Costa Rica

2020 ◽  
Vol 60 (3) ◽  
Author(s):  
Adriana Porras Moreno ◽  
Danny Ugalde Solera
Keyword(s):  
Factor Viii ◽  
Immune Tolerance ◽  
Hemophilia A ◽  
Tolerance Induction ◽  
Low Doses ◽  
Severe Hemophilia ◽  
Immune Tolerance Induction ◽  
On Demand ◽  
Hemophilic Patient ◽  
The World

Hemophilia A is an X – linked bleeding disorder. It can be treated with Factor VIII prophylaxis or on demand treatment. Most countries in the world use prophylaxis as it is less expensive than treating patients when they are bleeding. The production of factor VIII inhibitors is the most common and serious complication of the treatment. Immune tolerance induction (ITI) is the only option of treatment when patients develop inhibitors proven to be successful to eradicate this condition, therefore decreasing inhibitors and costs. A case of a successful immune tolerance induction with low doses of factor VIII (FVIII) in a pediatric patient with severe hemophilia A and FVIII inhibitors is presented. Even though inmunotolerance has been practice before in our country, a case like this has never been published.

scholarly journals What is the Evidence for the Use of Immunomodulatory Agents to Eradicate Inhibitory Antibodies in Patients with Severe Hemophilia A Who Have Previously Failed to Respond to Immune Tolerance Induction?

Hematology ◽  
2011 ◽  
Vol 2011 (1) ◽  
pp. 405-406 ◽  
Author(s):  
Michael U. Callaghan ◽  
Patrick F. Fogarty
Keyword(s):  
Immune Tolerance ◽  
Hemophilia A ◽  
High Titer ◽  
Tolerance Induction ◽  
High Dose ◽  
Severe Hemophilia ◽  
Inhibitory Antibody ◽  
Immunomodulatory Agents ◽  
Immune Tolerance Induction ◽  
Inhibitory Antibodies

Abstract An 18-year-old man has severe hemophilia A that has been complicated by a high-titer inhibitory antibody (peak 170 BU/mL). He had previously failed a trial of immune tolerance induction (ITI) using daily high-dose (100 units/kg/d) factor VIII (FVIII) for 20 months and would like to know if immunomodulatory agents, with or without another course of ITI, might eradicate the inhibitor.

Benefits of prophylactic emicizumab in enhancing immune tolerance induction in a boy with hemophilia A and very high inhibitor titer

2021 ◽  
Author(s):  
Nongnuch Sirachainan ◽  
Ampaiwan Chuansumrit ◽  
Surapan Parapakpenjune ◽  
Pakawan Wongwerawattanakoon ◽  
Surapong Lertthammakiat ◽  
...  
Keyword(s):  
Immune Tolerance ◽  
Hemophilia A ◽  
Tolerance Induction ◽  
Immune Tolerance Induction ◽  
Inhibitor Titer ◽  
Very High

scholarly journals IgG subclasses of anti-FVIII antibodies during immune tolerance induction in patients with hemophilia A

2008 ◽  
Vol 142 (4) ◽  
pp. 644-652 ◽  
Author(s):  
Pauline M. W. van Helden ◽  
H. Marijke van den Berg ◽  
Samantha C. Gouw ◽  
Paul H. P. Kaijen ◽  
Marleen G. Zuurveld ◽  
...  
Keyword(s):  
Immune Tolerance ◽  
Hemophilia A ◽  
Tolerance Induction ◽  
Igg Subclasses ◽  
Immune Tolerance Induction
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