Acquired haemophilia: an easy diagnosis to miss in a patient taking warfarin

2015 ◽  
Vol 14 (3) ◽  
pp. 122-124
Author(s):  
Avraneel Talapatra ◽  
◽  
Michael J. Nash ◽  
Charles R. M. Hay ◽  
Jecko Thachil ◽  
...  
Keyword(s):  
Factor Viii ◽  
Warfarin Therapy ◽  
Soft Tissues ◽  
Bleeding Disorder ◽  
Spontaneous Bleeding ◽  
Acquired Haemophilia

Acquired Haemophilia (AH) is an autoimmune bleeding disorder, which despite being rare, can be fatal. It occurs in patients with previously normal haemostasis who spontaneously develop IgG autoantibodies against factor VIII. Unlike congenital haemophilia, it manifests as spontaneous bleeding into skin and soft tissues. The presentation can be masked in patients who are receiving warfarin where the bleeding is often attributed to warfarin therapy, as in the case described in this report. Consideration of AH is important in patients taking anticoagulants, when coagulopathy and bleeding fails to correct with usual measures.

scholarly journals Progressive Intramuscular Haematoma in a 12-Year-Old Boy: A Case of Acquired Haemophilia A

2018 ◽  
Vol 2018 ◽  
pp. 1-3
Author(s):  
Manori Gamage ◽  
Sadeepa Weerasinghe ◽  
Mohamed Nasoor ◽  
A. M. P. W. Karunarathne ◽  
Sashi Praba Abeyrathne
Keyword(s):  
Factor Viii ◽  
Soft Tissues ◽  
Coagulation Factor ◽  
Bleeding Disorder ◽  
Haemophilia A ◽  
Fviii Inhibitor ◽  
Acquired Haemophilia ◽  
High Antibody Titer ◽  
History Of ◽  
The Right

Acquired hemophilia A (AHA) is a rare bleeding disorder due to acquired antibodies against coagulation factor VIII (FVIII). It is rare in children less than 16 years old, and the incidence is 0.45/million/year. An otherwise healthy, 12-year-old boy was admitted to the ward with a history of swelling of the right and left forearms, for 1 day duration. He did not have any history of trauma or bleeding disorder. He had prolonged APPTT level with very high antibody titer against factor VIII. His gene expression for factor VIII was found to be normal. He was managed with FEIBA and recombinant FVII activated complexes and prednisolone 1 m/kg/day regime to control bleeding. AHA is associated with several underlying pathologies such as pregnancy, autoimmune diseases, malignancy, medications and infections; however, up to 50% of reported cases are idiopathic. In contrast to congenital haemophilia A, in which haemarthrosis is the hallmark clinical presentation, patients with AHA mainly bleed in to the skin, muscles, and soft tissues. High mortality rate of more than 20% is either to retroperitoneal or intracranial bleeds. Diagnosis is confirmed on isolated prolongation of activated partial thromboplastin time which does not normalize after addition of normal plasma, reducing the factor VIII levels with evidence of FVIII inhibitor activity. They have normal prothrombin time and platelet functions. Management of AHA involves two aspects, namely, eradication of antibodies and maintaining effective haemostasis during a bleeding episode.

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.

scholarly journals Efficacy of Corticosteroids Alone in the Eradication of Factor VIII Inhibitor in an Old Female with Idiopathic Acquired Haemophilia A: Description of a Case

2012 ◽  
Vol 2012 ◽  
pp. 1-3
Author(s):  
Francesco Girelli ◽  
Chiara Biasoli ◽  
Bruna Bassi ◽  
Franco Bagioni ◽  
Gabriele Bondi ◽  
...  
Keyword(s):  
Factor Viii ◽  
Coagulation Factors ◽  
Personal History ◽  
Haemodynamic Instability ◽  
Factor Viii Inhibitor ◽  
Haemophilia A ◽  
Spontaneous Bleeding ◽  
Acquired Haemophilia ◽  
Clinical Challenge ◽  
Viii Inhibitor

Acquired haemophilia A (AHA) is a rare and serious disorder mainly affecting elderly patients. It is caused by the production of autoantibodies directed against coagulation factors; patients present with spontaneous bleeding, potentially fatal, in the absence of familial or personal history. Autoimmune disorders, infections, solid and hematologic tumors, and drugs are predisposing factors, but up to 50 percent of cases remain unexplained. The diagnosis of AHA is confirmed by specific laboratory tests; and the therapy is a clinical challenge, due to the fact that older patients are often affected by comorbidities. By passing agents may be used when persistent bleeding or haemodynamic instability is observed; corticosteroids, alone or with immunosuppressive therapy, are necessary to inhibit the production of the autoantibodies. We describe a case in which steroids in monotherapy successfully, safely, and persistently inhibited the production of anti-Factor VIII antibodies, in an old patient admitted after rheumatologic consult.

Acquired Haemophilia: Functional Study of Antibodies to Factor VIII

1981 ◽  
Vol 45 (03) ◽  
pp. 285-289 ◽  
Author(s):  
J P Allain ◽  
A Gaillandre ◽  
D Frommel
Keyword(s):  
Factor Viii ◽  
Functional Study ◽  
Factor Viii Inhibitor ◽  
Acquired Haemophilia ◽  
Viii Inhibitor ◽  
Kinetics Of ◽  
Antibody Function ◽  
Native Plasma

SummaryFactor VIII complex and its interaction with antibodies to factor VIII have been studied in 17 non-haemophilic patients with factor VIII inhibitor. Low VIII:C and high VIIIR.Ag levels were found in all patients. VIII:WF levels were 50% of those of VTIIRrAg, possibly related to an increase of poorly aggregated and electrophoretically fast moving VIIIR:Ag oligomers.Antibody function has been characterized by kinetics of VIII :C inactivation, saturability by normal plasma and the slope of the affinity curve. Two major patterns were observed:1) Antibodies from 6 patients behaved similarly to those from haemophiliacs by showing second order inhibition kinetics, easy saturability and steep affinity slope (> 1).2) Antibodies from other patients, usually with lower titres, inactivated VIII :C according to complex order kinetics, were not saturable, and had a less steep affinity slope (< 0.7). In native plasma, or after mixing with factor VIII concentrate, antibodies of the second group did not form immune complexes with the whole factor VIII molecular complex. However, dissociation procedures did release some antibodies from apparently low molecular weight complexes formed in vivo or in vitro. For appropriate management of non-haemophilic patients with factor VIII inhibitor, it is important to determine the functional properties of their antibodies to factor VIII.

P 736. Benign Enlargement of Subarachnideal Spaces and Subdural Hematoma in an Infant: Spontaneous Bleeding, Child Abuse, or Bleeding Disorder?

2018 ◽  
Author(s):  
Kristine Adam ◽  
Leo Rossler ◽  
Christine Decker ◽  
Charlotte Thiels ◽  
Christoph Heyer ◽  
...  
Keyword(s):  
Child Abuse ◽  
Subdural Hematoma ◽  
Bleeding Disorder ◽  
Spontaneous Bleeding

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

scholarly journals Haemophilic arthropathy

2014 ◽  
Vol 2 (4) ◽  
pp. 225-227
Author(s):  
Sarath Chandra Mouli V ◽  
Pradeep H
Keyword(s):  
Factor Viii ◽  
Lupus Erythematosus ◽  
Treatment Options ◽  
Left Groin ◽  
Left Hand ◽  
Acquired Haemophilia ◽  
Haemophilic Arthropathy ◽  
Fatal Bleeding ◽  
Coagulant Activity ◽  
History Of

Acquired haemophilia (AH) is extremely rare and potentially fatal bleeding disorder and it is associated with significant morbidity and mortality rate between 7.9% to 22%. Upto 90% of people with AH experience bleeding. It is mainly due to the spontaneous development of auto antibodies that inhibit the coagulant activity of factor VIII (FVIII). Autoimmune disorders (16.6%) like Rheumatoid arthritis, Systemic lupus erythematosus (SLE), Multiple Sclerosis etc., may lead to trigger acquired inhibitors to Factor VIII. In the case report we described a 70-year-old male patient who has history of migratory joint pains and swellings for five months and on physical examination there was an echymotic rash over the left hand elbow and left groin extending into the left thigh and we reemphasize that even though a series of effective treatment options available, a through workup, early diagnosis and early treatment are important.

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
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