Anticoagulation for Stroke Prevention after Restoration of Haemostasis with Emicizumab in Acquired Haemophilia A

Abstract

Acquired haemophilia A (AHA) is a rare haemorrhagic disorder caused by the development of autoantibodies inhibiting factor VIII function. It predominantly affects the elderly, who are often burdened with a considerable number of comorbidities, and can result in life-threatening bleeding. The management of AHA consists of two aspects: inhibitor eradication with an immunomodulator and bleed control with a bypassing agent. Here we present a case of AHA with a high titre inhibitor in a patient with extensive comorbidities and atrial fibrillation in whom inhibitor eradication could not be achieved within a few weeks using corticosteroids alone. Due to coronavirus disease (COVID)-19 restrictions and complications of care, emicizumab offered an effective and convenient therapy, not only sparing the need for continued and intensified inhibitor eradication, but also allowing anticoagulation for stroke prophylaxis.

Acquired hemophilia in patients on oral anticoagulant therapy: Case report

“…At the time of diagnosis, the patient had life-threatening bleeding in the oropharynx, which was provoked by gastroscopy. Delays in the diagnosis of acquired hemophilia leading to frequently fatal bleeding, in patients on oral anticoagulant therapy, have also been reported in literature [13][14][15]. Any invasive diagnostics or surgical procedure in patients with unrecognized acquired hemophilia can provoke heavy bleeding, as was the case with our patient [6,8,18].…”

“…U trenutku kada je postavljena dijagnoza, bolesnik je imao po život opasno krvarenje u orofarinksu, koje je bilo isprovocirano gastroskopijom. I u literaturnim podacima se navodi kašnjenje u dijagnozi stečene hemofilijie kod bolesnika na oralnoj antikoagulantnoj terapiji, što dovodi do često fatalnih krvarenja [13][14][15]. Svaka invazivna dijagnostika ili hirurška intervencija kod bolesnika sa neprepoznatom stečenom hemofilijom može isprovocirati obilna krvarenja, kao što je bio slučaj kod našeg bolesnika [6,8,18].…”

“…novel oral anticoagulants -NOACs) -dabigatran, rivaroksaban, apiksaban, važno je napomenuti da su i kod upotrebe ovih lekova opisani slučajevi stečene hemofilije [16,17]. Prilikom ordiniranja NOAC-a obično se ne kontroliše koagulacioni status, ali je značajno naglasiti da vrednosti INR-a i aPTT-a, takođe mogu biti u manjoj meri izmenjeni [5][6][7][8][9][10][11][12][13][14][15][16][17]23]. Stoga, svaku pojavu krvarenja, posebno mukokutanog, kod bolesnika na terapiji NOAC-ima treba propratiti novim analizama INR-a i aPTT-a.…”

“…Obično se nivo inhibitora postepeno smanjuje tokom više narednih meseci [6,8,[10][11]. Kod bolesnika koji su na oralnoj antikoagulantnoj terapiji a imaju hemoragijski sindrom ovo stanje se može prevideti, jer se obično kontroliše samo protrombinsko vreme [12][13][14][15].…”

“…Usually, the level of inhibitors gradually decreases over the following months [6,8,[10][11]. In patients on oral anticoagulant therapy who have hemorrhagic syndrome, this condition can be overlooked, since, usually, only the prothrombin time is monitored [12][13][14][15].…”