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

Acquired haemophilia A treated with recombinant porcine factor VIII: a single centre UK experience

2020 ◽  
Vol 189 (4) ◽  
Author(s):  
Dalia Khan ◽  
Sayma Raza‐Burton ◽  
Peter Baker ◽  
Sarah Harper ◽  
James Beavis ◽  
...  
Keyword(s):  
Factor Viii ◽  
Haemophilia A ◽  
Single Centre ◽  
Acquired Haemophilia

Does Peroral Administration of Factor VIII Induce Oral Tolerance in Patients with Acquired Haemophilia A?

2000 ◽  
Vol 83 (04) ◽  
pp. 632-633 ◽  
Author(s):  
Hans Wadenvik ◽  
Andrej Tarkowski ◽  
Lilian Tengborn ◽  
Arne Lindgren
Keyword(s):  
Factor Viii ◽  
Oral Tolerance ◽  
Peroral Administration ◽  
Haemophilia A ◽  
Acquired Haemophilia

scholarly journals Emicizumab in acquired haemophilia A: about two clinical cases and literature review

2021 ◽  
Vol 12 ◽  
pp. 204062072110381
Author(s):  
Amandine Hansenne ◽  
Cedric Hermans
Keyword(s):  
Autoimmune Disease ◽  
Literature Review ◽  
Factor Viii ◽  
Immunosuppressive Therapy ◽  
Current Literature ◽  
Mode Of Action ◽  
Bispecific Antibody ◽  
Haemophilia A ◽  
Acquired Haemophilia ◽  
Excess Morbidity

Acquired haemophilia A (AHA) is a rare and severe haemorrhagic autoimmune disease caused by autoantibodies directed against factor VIII (FVIII). Treatment is based on two principles, including haemostatic control to compensate FVIII inhibition and eradication of inhibiting antibodies using immunosuppressive therapy. Rapid recognition and proper management are essential to avoid excess morbidity and mortality. Effective and safe treatments can be challenging, given that AHA patients are often elderly, with multiple comorbidities. Emicizumab, a bispecific antibody that mimics the action of FVIII, has proven effective in managing patients with congenital haemophilia, with or without inhibitors. Likewise, its mode of action suggests theoretical efficacy in AHA patients. We herein describe two AHA cases with comorbidities that were treated effectively using emicizumab combined with immunosuppressive therapy. We have also reviewed the current literature regarding the promising use of emicizumab in this indication.

Acquired haemophilia a secondary to multiple myeloma: management of a patient with a mechanical mitral valve

2020 ◽  
Vol 13 (9) ◽  
pp. e230798
Author(s):  
Lisa B Pinchover ◽  
Rami Alsharif ◽  
Talia Bernal
Keyword(s):  
Multiple Myeloma ◽  
Mitral Valve ◽  
Factor Viii ◽  
Primary Care Doctor ◽  
Factor Viii Inhibitor ◽  
Haemophilia A ◽  
Acquired Haemophilia ◽  
Viii Inhibitor ◽  
Spontaneous Haematoma ◽  
Acquired Factor Viii Inhibitor

A 77-year-old man with a mechanical mitral valve on warfarin presented with an acute drop in haemoglobin and large spontaneous haematoma. He was found to have a new coagulopathy with initial labs notable for a prolonged activated partial thromboplastin time (APTT). Further workup revealed factor VIII levels less than 1%, abnormal mixing studies and elevated Bethesda titres, which was consistent with an acquired factor VIII inhibitor. Given his bone marrow biopsy result, which was positive for plasma cell myeloma, this coagulopathy was thought to be an acquired haemophilia A secondary to multiple myeloma. Anticoagulation was a challenge in this patient given his mechanical mitral valve and acquired haemophilia A. Although the patient was at risk of thrombosis due to a mechanical mitral valve, he had a bleeding diathesis and anaemia not responsive to transfusion. The decision was made to hold anticoagulation and the patient was started on myeloma treatment which included CyBorD, rituximab and daratumumab. After initiation of treatment APTT and factor VIII normalised. He eventually restarted anticoagulation under direction of his primary care doctor.

Factor VIII Inhibitors in Mild and Moderate-severity Haemophilia A

1998 ◽  
Vol 79 (04) ◽  
pp. 762-766 ◽  
Author(s):  
C.A. Ludlam ◽  
B.T. Colvin ◽  
F.G.H. Hill ◽  
F.E. Preston ◽  
N. Wasseem ◽  
...  
Keyword(s):  
Factor Viii ◽  
Tolerance Induction ◽  
Haemophilia A ◽  
Missense Mutations ◽  
Bleeding Pattern ◽  
Spontaneous Bleeding ◽  
Familial Predisposition ◽  
Inhibitor Development ◽  
Acquired Haemophilia ◽  
History Of

SummaryTwenty six patients with mild or moderate haemophilia A and inhibitors are described. The inhibitor was detected at a median age of 33 years, after a median of 5.5 bleeding episodes. This usually following intensive replacement therapy. The median presenting inhibitor titre was antihuman 11.6 BU/ml, antiporcine 1.45 BU/ml. Plasma basal factor VIII level declined from a median of 0.08 IU/ml to 0.01 IU/ml following the inhibitor development. This caused spontaneous bleeding in 22 and a bleeding pattern similar to acquired haemophilia in 17. Bleeding was often severe and caused two deaths.The inhibitor disappeared spontaneously, or following immune tolerance induction, in 16 cases after a median of 9 months (range 0.5-46), with a return to the original baseline VIIIC level and bleeding pattern accompanied inhibitor loss. The inhibitor persisted in the remainder of the cases over a median period of 99 months (range 17-433 months) of follow-up. Inhibitors are an uncommon complication of mild haemophilia which frequently persist and may be associated with severe, life-threatening, haemorrhage.Forty-one percent of treated haemophilic family members had a history of factor VIII inhibitors, suggesting a familial predisposition to develop inhibitors in these kindreds. Sixteen patients from 11 families were genotyped. Seven different missense mutations affecting the light chain were detected and two in the A2 domain. Five patients from three families had a mutation causing a substitution of Trp2229 by Cys in the C2 domain which appears to predispose to inhibitor formation since 7 of the 18 affected individuals have a history of inhibitor development.

A successful physiotherapy management case of a patient with acquired haemophilia A prior to factor VIII inhibitor eradication

Haemophilia ◽  
2016 ◽  
Vol 22 (3) ◽  
pp. e228-e231 ◽  
Author(s):  
M. Goto ◽  
N. Haga ◽  
K. Yokota ◽  
K. Takamizawa ◽  
H. Takedani
Keyword(s):  
Factor Viii ◽  
Factor Viii Inhibitor ◽  
Haemophilia A ◽  
Acquired Haemophilia ◽  
Viii Inhibitor

scholarly journals Recombinant porcine sequence factor VIII (rpFVIII) for acquired haemophilia A: practical clinical experience of its use in seven patients

Haemophilia ◽  
2016 ◽  
Vol 23 (1) ◽  
pp. 25-32 ◽  
Author(s):  
M. D. Tarantino ◽  
A. Cuker ◽  
B. Hardesty ◽  
J. C. Roberts ◽  
M. Sholzberg
Keyword(s):  
Factor Viii ◽  
Clinical Experience ◽  
Haemophilia A ◽  
Acquired Haemophilia

Factor VIII inhibitor eradication with bortezomib in acquired haemophilia A

2016 ◽  
Vol 178 (6) ◽  
pp. 986-987 ◽  
Author(s):  
James D. McFadyen ◽  
Huyen Tran ◽  
Zane S. Kaplan
Keyword(s):  
Factor Viii ◽  
Factor Viii Inhibitor ◽  
Haemophilia A ◽  
Acquired Haemophilia ◽  
Viii Inhibitor
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