International recommendations on the diagnosis and treatment of patients with acquired hemophilia A

A case of acquired hemophilia A in an elderly female

“…Other common causes are heparin therapy or lupus anticoagulant [9,10]. The next step is to perform a mixing study, in which the patient’s plasma is combined with normal plasma in a 1:1 ratio and incubated at 37 °C for at least an hour.…”

“…After confirming the presence of the inhibitor, the Bethesda assay is used to evaluate inhibitor titer levels. Neither factor VIII activity nor inhibitor titer correlates well with the disease activity or severity of bleeding [10]. …”

“…There are no randomized control data to guide us on management, and most of the data is derived from observational data from the EACH2 registry and from treatment of patients with Congenital Hemophilia with or without inhibitors. International practice guidelines have been derived by Huthe-Kuhne et al, based on analysis of the data available and clinical experience [10]. …”

“…rFVIIa has been noted to be effective/partially effective in 90% nonsurgical and 86% surgical cases [12]. Some studies have reported it to have an effective response in 100% cases when used as first line therapy, 75% good or partial response as second line therapy, and 17% response when used as salvage therapy [10,13]. aPCC is an alternative to rVIIa as a bypassing agent.…”

“…Factor eight inhibitor bypassing activity (FEIBA) is the only aPCC available in USA, containing activated factor VII along with non-activated factors II, IX, and X [14]. FEIBA has been known to have a response rate of 76% in severe bleeding and 100% in moderate bleeding as observed in a retrospective study [10,15]. Based on the data in the EACH2 registry, bypassing agents were superior when compared with desmopressin or factor VIII concentrates, however there is no head-to-head comparison of the efficacy of aPCC and rVIIa.…”