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.

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.

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.

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

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

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.

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.

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