Myocardial infarction after FEIBA therapy in a hemophilia-B patient with a factor IX inhibitor

Abstract

A case of myocardial infarction (MI) in a hemophilia B patient with a factor IX (FIX) inhibitor (6 BU) is described. MI occurred after two infusions of FEIBA concentrate. Unexpectedly, these infusions resulted in a neutralization of the inhibitor and a consistent plasma FIX activity which may have increased the thrombotic risks. Four days later, a psoas hematoma was suspected. At that time the inhibitor remained undetectable, allowing a therapy with purified FIX concentrates. No recurrence of thrombotic complication was observed. This is an additional illustration of the thrombotic risks associated with the use of activated prothrombin complex concentrates, especially in patients having pre-existing risk factors for thrombosis. The management of bleeding episodes in hemophilia B patients with inhibitor represents an especially difficult challenge.

Cases of surgery in high‐responder haemophilia patients

“…Finally in the case of the last patient (Case 4) FEIBA ® was used as first intention treatment for this major surgery because it has been largely used by the patient [1]. Haemorrhagic complications occurred approximately 12 h after the intervention, can be related to insufficient amounts of FEIBA ® in the post operative period and possibly also to LMWH.…”

“…This 52‐year‐old man was a high‐responding, severe haemophilia B patient with acute myocardial infarction antecedent that occurred while the patient was under FEIBA ® (Baxter, Deerfield, IL USA) therapy [1]. He also developed an anti‐erythrocyte antibody of very broad specificity (anti‐public) which made the selection of compatible red blood cells difficult.…”

Management of difficult‐to‐treat inhibitor patients

“…If bleeding continues, consider sequential FEIBA ® / NovoSeven ® according to the protocol of Schneiderman et al. [20], with focus on the risks of FEIBA ® use in FIX‐deficient patients [21,22]. Increase the dose of NovoSeven ® to 270 μg kg −1 plus TXA.…”

Identifying and managing inhibitor patients requiring orthopaedic surgery – the multidisciplinary team approach

“…Indeed, although aPCCs have been used successfully to treat bleeds in haemophilia patients with inhibitors, FEIBA TM has, in the past, been associated with thrombogenicity. This effect, albeit rare, appears to increase with increasing dose [1,12,13].…”