Acquired haemophilia A: A 2013 update

2013 ◽  
Vol 110 (12) ◽  
pp. 1114-1120 ◽  
Author(s):  
Massimo Franchini ◽  
Pier Mannucci
Keyword(s):  
Drug Exposure ◽  
Current Knowledge ◽  
Post Partum ◽  
Coagulation Factor ◽  
The Elderly ◽  
Bleeding Disorder ◽  
Severe Bleeding ◽  
Haemophilia A ◽  
Autoantibody Production ◽  
Acquired Haemophilia

SummaryAcquired haemophilia A (AHA) is a rare but often severe bleeding disorder caused by autoantibodies against coagulation factor VIII (FVIII). AHA occurs more frequently in the elderly and in association with several conditions, such as the post-partum period, malignancies, autoimmune diseases or drug exposure; however, approximately 50% of reported cases are apparently idiopathic. Beside the elimination of the underlying disorder, the therapeutic approach to AHA should be directed toward the control of acute bleed and the eradication of FVIII autoantibody production. In this narrative review, we summarise the current knowledge on the epidemiology, diagnosis and clinical features of AHA, focusing in particular on advances in the management of this challenging bleeding disorder.

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.

Acquired haemophilia A diagnosed during pregnancy

2021 ◽  
pp. 1753495X2110499
Author(s):  
Ayesha Ejaz ◽  
Claire O’Doherty ◽  
Faye A. Sharpley ◽  
Nicola Curry ◽  
Susan Shapiro ◽  
...  
Keyword(s):  
Post Partum ◽  
Bleeding Disorder ◽  
Haemophilia A ◽  
Consensus Guidelines ◽  
Referral Centre ◽  
Tertiary Referral ◽  
Tertiary Referral Centre ◽  
Acquired Haemophilia ◽  
Other Woman ◽  
In Pregnancy

Pregnancy-associated haemophilia A is an uncommon, acquired bleeding disorder which usually presents post-partum; very rarely it may present during pregnancy. No consensus guidelines exist on the management of this condition in pregnancy and very few cases have been reported in the literature. Here we describe the case of a woman presenting with acquired haemophilia A during pregnancy and outline the management of her bleeding disorder. We contrast her case with that of two other woman, presenting to the same tertiary referral centre, with acquired haemophilia A presenting post-partum. These cases highlight the heterogeneous management of this condition and how it may be successfully managed in pregnancy.

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.

scholarly journals Acquired Haemophilia A. Which is the best therapeutic choice in older adults? Single center study of 4 cases

Reumatismo ◽  
2019 ◽  
Vol 71 (1) ◽  
pp. 37-41 ◽  
Author(s):  
E. Mauro ◽  
E. Garlatti Costa ◽  
A. Zanier ◽  
M. Maset ◽  
A. Ermacora ◽  
...  
Keyword(s):  
Coagulation Factor ◽  
Factor Vii ◽  
Haemophilia A ◽  
Recombinant Activated Factor Vii ◽  
Acquired Haemophilia ◽  
Activated Factor Vii ◽  
Activated Prothrombin Complex Concentrate ◽  
Therapeutic Choice ◽  
Single Center Study ◽  
Thrombotic Complications

Acquired haemophilia A (AHA) is a rare bleeding disorder due to autoantibodies directed against coagulation factor VIII. The treatment is based on recombinant activated factor VII and activated prothrombin complex concentrate. However, mainly in older patients, severe thrombotic complications have been reported. Here we report the different therapeutic approaches in 4 cases of elderly patients with AHA and co-morbidities.

scholarly journals Acquired Haemophilia A in Association with Influenza A and Urinary Tract Infection

2020 ◽  
Author(s):  
Felipe Peña-Muñoz ◽  
Ernesto Parras ◽  
Olga Compan ◽  
Nora Gutierrez ◽  
Celestino Martin ◽  
...  
Keyword(s):  
Influenza A ◽  
Coagulation Factor ◽  
Autoimmune Disorder ◽  
Immunosuppressive Drugs ◽  
Bleeding Complications ◽  
Haemophilia A ◽  
Acquired Haemophilia ◽  
Haemostatic Agents ◽  
Inhibitor Titre ◽  
Prolonged Aptt

Acquired haemophilia A (AHA) is a rare autoimmune disorder caused by an autoantibody against any circulating coagulation factor, especially factor VIII (FVIII). The lack of awareness of this condition suggests that diagnosis is a challenge and usually delayed, which leads to suboptimal treatment. Consequently, early diagnosis is mandatory to prevent potentially life-threatening bleeding complications. We present the case of an 85-year-old woman admitted to hospital with symptoms of respiratory infection who 12 hours later developed haematuria which required transfusion. Laboratory assays showed an isolated prolonged aPTT, a moderately reduced FVIII and a high inhibitor titre. Influenza A and Escherichia coli were also identified. Antivirals, antibiotics, immunosuppressive drugs and haemostatic agents were started. Two weeks later, the inhibitor was not detected, and bleeding and symptoms of infection had resolved. Immunosuppressive drugs were stopped on day 45 and there has been no recurrence since then. To date, no FVIII inhibitors have been reported in concomitant infection with influenza A and urinary E. coli. The identification of conditions potentially associated with AHA is essential to achieve complete remission.

scholarly journals Acquired haemophilia A: the importance of early recognition in cases of spontaneous bleeding in the elderly

2014 ◽  
Vol 2014 (nov20 1) ◽  
pp. bcr2014206911-bcr2014206911 ◽  
Author(s):  
N. Patel ◽  
Z. Wyrko ◽  
S. Naqvi ◽  
A. P. Croft
Keyword(s):  
Early Recognition ◽  
The Elderly ◽  
Haemophilia A ◽  
Spontaneous Bleeding ◽  
Acquired Haemophilia

scholarly journals SUCCESSFUL LONG TERM ERADICATION OF FACTOR VIII INHIBITOR IN PATIENTS WITH ACQUIRED HAEMOPHILIA A IN SAUDI ARABIA

2012 ◽  
Vol 4 (1) ◽  
pp. e2012021 ◽  
Author(s):  
Galila F Zaher ◽  
Soheir S Adam
Keyword(s):  
Coagulation Factor ◽  
Complex Nature ◽  
Factor Viii Inhibitor ◽  
Haemophilia A ◽  
Acquired Haemophilia ◽  
Local Experience ◽  
Fatal Bleeding ◽  
Factor Inhibitor ◽  
Initial Control ◽  
Initiation Of Therapy

Acquired haemophilia A is a serious and potentially fatal bleeding disorder. Diagnosis is difficult and maybe delayed due to its rarity. The high mortality rate and the complex nature of treatment necessitate patient management at a haemophilia centre, where the required expertise and resources are available. Prompt diagnosis is crucial and early initiation of therapy could be life saving. Management includes initial control of bleeding followed by an approach to eradicate the coagulation factor inhibitor. In this paper we describe our local experience with acquired haemophilia A, which resulted in the successful control of major bleeding at presentation and eradication of inhibitors.

A szerzett haemophilia A sikeres kezelése

2021 ◽  
Vol 162 (49) ◽  
pp. 1977-1981
Keyword(s):  
Partial Thromboplastin Time ◽  
Early Recognition ◽  
Coagulation Factor ◽  
Autoimmune Disorder ◽  
Severe Anaemia ◽  
Rapid Diagnosis ◽  
Activated Partial Thromboplastin Time ◽  
Haemophilia A ◽  
Adequate Treatment ◽  
Acquired Haemophilia

Összefoglaló. A szerzett haemophilia A ritka autoimmun betegség, melyben gátlótest képződik a VIII. véralvadási faktor ellen. Az inhibitor véralvadásra gyakorolt hatása súlyos, életet veszélyeztető vérzéses állapotot idéz elő. A beteg élete a gyors diagnózison múlik: a jellemző klinikai kép mellett a megnyúlt, normálplazmával nem korrigálható aktivált parciális tromboplasztinidő megléte esetén a kórkép alapos gyanúja merül fel. Egy súlyos vérszegénység miatt kórházunkba beutalt nőbeteg esetében a szerzett haemophilia A a felvételt követő napon már diagnosztizálásra került. A vérzés megszüntetésére aktivált protrombinkomplex-koncentrátumot alkalmaztunk, valamint immunszuppresszív terápiát vezettünk be. A kórkép korai felismerése és a megfelelő kezelés azonnali megkezdése a beteg gyógyulását eredményezte. Esetünkkel arra szeretnénk felhívni a figyelmet, hogy a szerzett haemophilia A gyors diagnózisa egyszerű, könnyen hozzáférhető véralvadási paraméter, az aktivált parciális tromboplasztinidő meghatározásán és nem korrigálható megnyúlásának felismerésén múlik. Orv Hetil. 2021; 162(49): 1977–1981. Summary. Acquired haemophilia A is a rare autoimmune disorder, in which antibodies are formed against coagulation factor VIII. The effect of the inhibitor on blood clotting results in severe, life-threatening bleeding diathesis. The patient’s life depends on the rapid diagnosis: besides the characteristic clinical presentation, a prolonged activated partial thromboplastin time, which is not corrigible with normal plasma, suggests the existence of the disorder. In the case of the female patient who was referred to our hospital due to severe anaemia, acquired haemophilia A was diagnosed rapidly, the day after her admission. We used activated prothrombin complex concentrate to stop the bleeding, and introduced immunosuppressive therapy. The early recognition of the disease and immediate initiation of adequate treatment resulted in the patient’s full recovery. With our case presentation, we would like to draw attention to the fact that the rapid diagnosis of acquired haemophilia A depends on the determination of a simple, easily accessible coagulation parameter, the activated partial thromboplastin time and on the immediate recognition of its incorrigible prolongation. Orv Hetil. 2021; 162(49): 1977–1981.

Management of acquired haemophilia A

2015 ◽  
Vol 35 (04) ◽  
pp. 311-318 ◽  
Author(s):  
R. E. Scharf ◽  
C. Dobbelstein ◽  
S. Werwitzke ◽  
A. Tiede
Keyword(s):  
Factor Viia ◽  
Immunosuppressive Treatment ◽  
Coagulation Factor ◽  
Cytotoxic Agents ◽  
Haemophilia A ◽  
Recent Onset ◽  
Acquired Haemophilia ◽  
First Line Therapy ◽  
Characteristic Features ◽  
Bypassing Agents

SummaryAcquired haemophilia A (AHA) is caused by autoantibody inhibitors of coagulation factor VIII (FVIII : C). Recent onset of bleeds and isolated prolongation of the activated partial thromboplastin time (aPTT) are characteristic features of the disorder. Reduced FVIII : C activity and a detectable FVIII : C inhibitor in the Bethesda assay confirm the diagnosis. Patients should be referred to expert centres, whenever possible, and invasive procedures with a high risk of bleeding must be avoided, until haemostasis has been secured by adequate therapy.Bypassing agents capable of inducing sufficient thrombin formation in the presence of FVIII : C inhibitors are treatment of choice, including currently available recombinant factor VIIa (NovoSeven™) and activated prothrombin complex concentrate (FEIBA™). These agents represent first line therapy to control acute or severe bleeds. To eradicate inhibitors, immunosuppressive treatment (IST) is indicated in patients with AHA. Glucocorticoids, cytotoxic agents and rituximab are most widely used. However, an ideal IST regimen has not been established so far. Adverse events of IST, including infections as the foremost cause death, are frequent complications in AHA.

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