Acquired hemophilia A

Pandey, Yadav; Atwal, Dinesh; Konda, Manojna; Roy, Arya Mariam; Sasapu, Appalanaidu

doi:10.1080/08998280.2019.1689024

Cited by 4 publications

(4 citation statements)

References 21 publications

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“…The existence of autoantibodies is not the same as the presence of autoimmune diseases; autoantibodies may exist in normal people without autoimmune diseases. A stress state can lead to the production of autoantibodies, but such antibodies have no pathogenic effect and belong to a secondary immune response ( 25 , 26 ). The present patient suffered from rectal leiomyoma before admission, which was a benign tumor and had no significant association with AHA.…”

Section: Discussionmentioning

confidence: 99%

Repeated bleeding caused by acquired hemophilia A after endoscopic submucosal dissection: A case report and literature review

et al. 2023

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Endoscopic submucosal dissection (ESD) has been widely used in the treatment of gastrointestinal tract lesions, and hemorrhage is one of the most common complications. The aim of the present study was to investigate the clinical characteristics of hemorrhage after ESD in patients with acquired hemophilia A (AHA). Firstly, a case of AHA with multiple bleeding events after ESD is reported. Colonoscopy was used to perform ESD treatment of the submucosal tumor, and immunohistochemical analysis was used to analyze the tumor properties. Secondly, literature relevant to postoperative hemorrhage caused by AHA was researched and analyzed, with the changes in activated partial thromboplastin time (APTT) before and after operation, coagulation factor VIII (FVIII) activity, FVIII inhibitor value and treatment plan noted. The majority of patients with AHA had no history of coagulation disorder or genetic disease and showed a normal APTT. However, it was found that the APTT value gradually increased after bleeding. In addition, the APTT correction test did not correct for prolonged APTT and FVIII antibody positivity in AHA. There was no bleeding or bleeding tendency prior to surgery in patients with AHA. The study concludes that when repeated bleeding and a poor hemostatic effect occurs, it is necessary to be alerted to the possibility of AHA, as an early diagnosis is essential for effective hemostasis.

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Section: Discussionmentioning

confidence: 99%

Repeated bleeding caused by acquired hemophilia A after endoscopic submucosal dissection: A case report and literature review

et al. 2023

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“…A szerzett haemophilia A életet veszélyeztető betegség [1], melynek során antitest képződik a FVIII ellen [9]. A veleszületett haemophiliában található alloantitestekhez hasonlóan a FVIII-ellenes autoantitestek is poliklonálisak, és az IgG1 és IgG4 alosztályokhoz tartoznak [10].…”

Section: Megbeszélésunclassified

“…A szerzett haemophilia ritka autoimmun betegség (1,5/ millió lakos/év), melyet faktorellenes antitestek megjelenése és spontán, életet veszélyeztető vérzések kialakulása jellemez [1,2]. A leggyakrabban a VIII.…”

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A szerzett haemophilia A sikeres kezelése

Kovács

Tajti

Szoboszlay

et al. 2021

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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.

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“…AHA is seen associated with malignancy, autoimmune diseases, pregnancy, drugs, and blood transfusions. However, up to 50% of reported cases remain idiopathic [5]. Unlike joint bleeds that characterize congenital hemophilia A, AHA manifests as spontaneous hematomas and extensive bruising, epistaxis, rarely GIB, and intracranial hemorrhage may occur [3,5,6].…”

Section: Introductionmentioning

confidence: 99%

Undiagnosed Acquired Hemophilia A: Presenting as Recurrent Gastrointestinal Bleeding

Roy¹,

Siddiqui

Venkata³

2020

Cureus

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Acute gastrointestinal bleeding (GIB) is a frequently encountered medical emergency and it can be lifethreatening depending on the etiology and the clinical condition of the patient. The most common causes of GIB are peptic ulcer disease, aspirin-induced gastritis, variceal hemorrhage, esophagitis, neoplasms like gastric cancer. Acquired hemophilia causing acute gastrointestinal bleed is extremely rare and only a few cases are reported worldwide. Acquired hemophilia A (AHA) is a rare disorder caused by the production of autoantibodies that inactivates clotting factor VIII. We present a case of upper gastrointestinal bleed due to AHA which was undiagnosed for two years. A 74-year-old patient with a history of myasthenia gravis, presented with anemia, and GIB. She underwent multiple endoscopies without a clear bleeding source. Coagulation studies showed isolated activated partial thromboplastin time prolongation which was not corrected by mixing study. Factor VIII activity was low and Bethesda titer showed elevated inhibitor levels. Factor Eight Bypassing Agent, recombinant factor VIIa, and steroids were given to control bleeding. Her clinical condition worsened, and she passed away. Elderly patients presenting with an undiagnosed source of GIBs, inconclusive endoscopic studies should be evaluated for acquired coagulopathies, especially in those with a history of autoimmune diseases and malignancies. Prompt diagnosis and treatment are warranted as it carries a high mortality.

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Acquired hemophilia A

Cited by 4 publications

References 21 publications

Repeated bleeding caused by acquired hemophilia A after endoscopic submucosal dissection: A case report and literature review

Repeated bleeding caused by acquired hemophilia A after endoscopic submucosal dissection: A case report and literature review

A szerzett haemophilia A sikeres kezelése

Undiagnosed Acquired Hemophilia A: Presenting as Recurrent Gastrointestinal Bleeding

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