scholarly journals Anticoagulation for Stroke Prevention after Restoration of Haemostasis with Emicizumab in Acquired Haemophilia A

2021 ◽  
Author(s):  
Kadhim Al-Banaa ◽  
Nicolas Gallastegui-Crestani ◽  
Annette von Drygalski
Keyword(s):  
Atrial Fibrillation ◽  
Factor Viii ◽  
Stroke Prevention ◽  
The Elderly ◽  
High Titre ◽  
Haemophilia A ◽  
Acquired Haemophilia ◽  
Inhibiting Factor ◽  
Life Threatening

Acquired haemophilia A (AHA) is a rare haemorrhagic disorder caused by the development of autoantibodies inhibiting factor VIII function. It predominantly affects the elderly, who are often burdened with a considerable number of comorbidities, and can result in life-threatening bleeding. The management of AHA consists of two aspects: inhibitor eradication with an immunomodulator and bleed control with a bypassing agent. Here we present a case of AHA with a high titre inhibitor in a patient with extensive comorbidities and atrial fibrillation in whom inhibitor eradication could not be achieved within a few weeks using corticosteroids alone. Due to coronavirus disease (COVID)-19 restrictions and complications of care, emicizumab offered an effective and convenient therapy, not only sparing the need for continued and intensified inhibitor eradication, but also allowing anticoagulation for stroke prophylaxis.

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

scholarly journals A case of serious bleeding

2015 ◽  
Vol 5 (2S) ◽  
pp. 15-19
Author(s):  
Irene Ricca ◽  
Marisa Coggiola ◽  
Silvia Destefanis ◽  
Claudio Pascale
Keyword(s):  
Mortality Rate ◽  
Autoimmune Diseases ◽  
Factor Viii ◽  
Coagulation Factor ◽  
The Elderly ◽  
Severe Anaemia ◽  
Severe Bleeding ◽  
Haemophilia A ◽  
Acquired Haemophilia ◽  
History Of

Acquired haemophilia A (AHA) is a rare disorder with a high mortality rate. It occurs due to autoantibodies against coagulation factor VIII (FVIII) which neutralise its procoagulant function resulting in severe bleeding. This disease may be associated with autoimmune diseases, malignancies, infections or medications and occurs most commonly in the elderly. Diagnosis is based on the isolated prolongation of aPTT which does not normalise after the addition of normal plasma along with reduced FVIII levels. Treatment involves eradication of antibodies and maintaining effective haemostasis during bleeding. We report a case of a 76-year-old patient with a history of haemorrhage with severe anaemia. The article describes difficulties and complexities of clinical and therapeutic management of the patient.

scholarly journals Acquired haemophilia – A diagnosis not to be missed

2008 ◽  
Vol 7 (2) ◽  
pp. 70-72
Author(s):  
S J Kitson ◽  
◽  
N F Grigoropoulos ◽  
Keyword(s):  
Autoimmune Diseases ◽  
Factor Viii ◽  
Coagulation Factor ◽  
Clinical History ◽  
Haemophilia A ◽  
Haematological Malignancies ◽  
Acquired Haemophilia ◽  
Wide Range ◽  
Life Threatening ◽  
History Of

Acquired haemophilia is a rare, life threatening bleeding disorder characterised by the development of auto-antibodies to coagulation factor VIII. Diagnosis is based upon the clinical history of mucocutaneous haemorrhages combined with a selective prolongation of the APTT. The condition is associated with a wide range of conditions, such as autoimmune diseases , solid and haematological malignancies. Treatment involves controlling the bleeding manifestations and eliminating the inhibitor antibodies. Three cases from our recent practice are used to highlight the variable severity of this condition.

Successful bleeding control with recombinant porcine factor VIII in reduced loading doses in two patients with acquired haemophilia A and failure of bypassing agent therapy

Haemophilia ◽  
2016 ◽  
Vol 22 (5) ◽  
pp. e472-e474 ◽  
Author(s):  
M. Stemberger ◽  
P. Möhnle ◽  
J. Tschöp ◽  
L. Ney ◽  
M. Spannagl ◽  
...  
Keyword(s):  
Factor Viii ◽  
Haemophilia A ◽  
Bleeding Control ◽  
Acquired Haemophilia

scholarly journals Acquired haemophilia A caused by inhibitor against factor VIII.

2001 ◽  
Vol 90 (6) ◽  
pp. 1085-1087
Author(s):  
Noriko Kakudo ◽  
Tomohiro Sugawara ◽  
Yasuhide Asaumi ◽  
Tatsuyuki Sato ◽  
Mitsushi Yano ◽  
...  
Keyword(s):  
Factor Viii ◽  
Haemophilia A ◽  
Acquired Haemophilia

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.

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