Successful control of bleeding during supracondylar amputation caused by severe compartment syndrome in patient with haemophilia A and high titre of inhibitor

Haemophilia ◽  
2009 ◽  
Vol 15 (2) ◽  
pp. 601-602 ◽  
Author(s):  
N. RAJIC ◽  
A. SAVIC ◽  
S. POPOVIC ◽  
I. UROSEVIC ◽  
I. SAVIC
Keyword(s):  
Compartment Syndrome ◽  
High Titre ◽  
Haemophilia A ◽  
Successful Control

Inhibitor treatment by rituximabin congenital haemophilia A

2009 ◽  
Vol 29 (02) ◽  
pp. 151-154 ◽  
Author(s):  
Escuriola Ettingshausen ◽  
R. Linde ◽  
G. Kropshofer ◽  
L.-B. Zimmerhackl ◽  
W. Kreuz ◽  
...  
Keyword(s):  
B Cell ◽  
Immune Tolerance ◽  
High Titre ◽  
Clotting Factor ◽  
Severe Bleeding ◽  
High Morbidity ◽  
Haemophilia A ◽  
Concomitant Treatment ◽  
Bleeding Tendency ◽  
Severe Haemophilia

SummaryThe development of neutralizing alloanti-bodies (inhibitors) to factor VIII (FVIII) is one of the most serious complications in the treatment of haemophiliacs. Inhibitors occur in approximately 20 to 30% of previously untreated patients (PUPs), predominantly children, with severe haemophilia A within the first 50 exposure days (ED). Immune tolerance induction (ITI) leads to complete elimination of the inhibitor in up to 80% of the patients and offers the possibility to restore regular FVIII prophylaxis. However, patients with high titre inhibitors, in whom standard ITI fails, usually impose with high morbidity and mortality and therefore prompting physicians to alternate therapy regimens. Rituximab, an anti-CD 20 monoclonal antibody has been successfully used in children and adults for the management of B-cell mediated disorders. We report on the use of a new protocol including rituximab in two adolescents with severe haemophilia A and high titre inhibitors, severe bleeding tendency and high clotting factor consumption after failing standard ITI. Both patients received a concomitant treatment with FVIII according to the Bonn protocol, cyclosporine A and immunoglobulin. Treatment with rituximab resulted in a temporary B-cell depletion leading to the disappearance of the inhibitor. FVIII recovery and half-life turned towards normal ranges. In patient 1 the inhibitor reappeared 14 months after the last rituximab administration. In patient 2 complete immune tolerance could be achieved for 60 months. Bleeding frequency diminished significantly and clinical joint status improved in both patients. In patient 1 the treatment course was complicated by aspergillosis and hepatitis B infection. Conclusion: Rituximab may be favourable for patients with congenital haemophilia, high-titre inhibitors and a severe clinical course in whom standard ITI has failed. Prospective studies are required to determine safety, efficacy and predictors of success.

Successful Control of Bleeding with Emicizumab in Aquired Haemophilia A: A Case Report

2019 ◽  
Author(s):  
S. Flommersfeld ◽  
J. Slonka ◽  
I. Bieberle ◽  
A. Stockschläder ◽  
B. Kemkes-Matthes ◽  
...  
Keyword(s):  
Case Report ◽  
Haemophilia A ◽  
Successful Control

Innowacyjna metoda prowadzenia ITI za pomocą preparatu czynnika VIII z frakcją Fc u pacjentów z hemofilią A z obecnością inhibitora czynnika VIII – przegląd literaturowy

2018 ◽  
Vol 22 (3) ◽  
Author(s):  
Maciej Trzaska ◽  
Marek Karwacki ◽  
Paweł Łaguna ◽  
Michał Matysiak
Keyword(s):  
Factor Viii ◽  
Tolerance Induction ◽  
Standard Of Care ◽  
Preliminary Evidence ◽  
Immunological Tolerance ◽  
Therapeutic Benefit ◽  
High Titre ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
First Time

Eradication of factor VIII inhibitors using Immune tolerance induction (ITI) treatment is the standard of care for severe haemophilia A patients presenting with factor VIII inhibitors, but is not always effective. A description of the potential immunological tolerance effect of the IgG Fc domain of recombinant factor VIII Fc fusion protein (rFVIIIFc), as well as published experience with rFVIIIFc for ITI in patients with severe haemophilia A and high-titre inhibitors. Review of published literature describing cases of ITI with rFVIIIFc in patients with severe haemophilia A and high-titre inhibitors between November 2015 and June 2018. Four publications has been found. Of 56 patients with haemophilia A who presented with FVIII inhibitors, 28 achieved a negative Bethesda titre (< 0.6) after ITI treatment using rFVIIIFc. Additional patients continued on rFVIIIFc ITI at the time of publication, while a few were reported to have switched to bypass therapy alone or other factors . For those still undergoing ITI, longer follow-up is needed to determine final outcomes. No adverse events were reported. Based on literature review, preliminary evidence of FVIIIFc use in high risk, first-time ITI suggests rapid time to tolerization. For rescue ITI, rFVIIIFc showed therapeutic benefit in some patients who previously failed ITI. These findings give hope but highlight the need for further evaluation in ongoing clinical trials.

scholarly journals A previously treated severe haemophilia A patient developed high-titre inhibitor after vaccinations

2020 ◽  
Vol 34 ◽  
pp. 205873842093461
Author(s):  
Zekun Li ◽  
Zhenping Chen ◽  
Xiaoling Cheng ◽  
Xinyi Wu ◽  
Gang Li ◽  
...  
Keyword(s):  
Neutralizing Antibody ◽  
High Titre ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Danger Signal ◽  
Inflammatory Microenvironment ◽  
Varicella Zoster ◽  
Close Proximity ◽  
Previously Treated ◽  
The Relationship

The factor VIII (FVIII)-neutralizing antibody (inhibitor) seen in 25%–30% of patients with severe haemophilia A (SHA). Vaccination is a non-genetic risk factor of inhibitor development as ‘danger signal’ which may provide a pro-inflammatory microenvironment to increase FVIII immunogenicity. We reported a previously treated SHA patient postponed the first vaccination to 15-month age received diphtheria-pertussis-tetanus intramuscularly. At 18-month age, the patient received Hepatitis A intramuscularly and Varicella Zoster Virus subcutaneously with 2 weeks interval and FVIII infusion was given <24 h prior for each. Successive bleedings occurred 1 week later with inefficacy of FVIII replacement. High-titre inhibitor was tested at 117 exposure days. This case suggested that continuous vaccinations in close proximity to FVIII could induce inhibitor. The relationship between vaccination and FVIII immunogenicity still needs to be revealed by further study.

One-stage replacement of infected knee prosthesis in a patient with haemophilia A and high titre of inhibitors

Haemophilia ◽  
2008 ◽  
Vol 14 (2) ◽  
pp. 375-377 ◽  
Author(s):  
R. SARTORI ◽  
R. BISSON ◽  
G. W. BAARS ◽  
U. PETRIS ◽  
P. RADOSSI ◽  
...  
Keyword(s):  
Knee Prosthesis ◽  
High Titre ◽  
Haemophilia A ◽  
One Stage

Daratumumab rapidly reduces high‐titre factor VIII inhibitors in haemophilia A patients during life‐threatening haemorrhages

Haemophilia ◽  
2020 ◽  
Author(s):  
Chatphatai Moonla ◽  
Noppacharn Uaprasert ◽  
Phandee Watanaboonyongcharoen ◽  
Mukmanee Meesanun ◽  
Autcharaporn Sukperm ◽  
...  
Keyword(s):  
Factor Viii ◽  
High Titre ◽  
Haemophilia A ◽  
Life Threatening
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