Optimal Management of an Aneurysmal Subarachnoid Hemorrhage in a Patient With Known Factor XI Deficiency: A Case Report

Abstract

The authors report a rare case of an acute cerebral aneurysm rupture in a patient with a known factor XI deficiency. Aneurysmal subarachnoid hemorrhage (SAH) accounts for a high mortality and morbidity rate. When SAH is associated with an inherited coagulation disorder such as hemophilia C, an unexpected and possible increase in hemorrhagic stroke and increase in bleeding during surgery and in the postoperative period could lead to an extremely bad outcome. Clinical management consists of rapid correction of the coagulation disorder before undergoing any invasive intracranial procedure. Such an optimal therapeutic strategy must be under the care of a multidisciplinary medical and surgical team. Human factor XI concentrate (Hemoleven, Laboratoire Français du Fractionnement et des Biotechnologies [LFB], Les Ulis, France) was used successfully in this case report. New treatment using recombinant factor VIIa is discussed.

Intracranial hemorrhage in adult patients with hematological malignancies

“…The current study examined prolonged prothrombin duration as one of the independent prognostic factors for ICH in patients with hematological malignancies. Leukemic cells may express fibrinolysis and other proteolysis enzymes, which has been implicated in the pathogenesis of bleeding in some patients [38-40]. Chemotherapy-related endothelial injury and reduction of coagulation factors also could play a role in the pathogenesis [38,39].…”

A surgical case of cerebral hemorrhage in a patient with factor XI deficiency

“…An optimal therapeutic strategy when one suffers from unanticipated bleeding is the administration of human FXI concentrate (HFC) [7]. However, infusion of HFC should be approached with caution, because fatal thrombosis events have been reported in some cases.…”

“…However, infusion of HFC should be approached with caution, because fatal thrombosis events have been reported in some cases. Siao et al [7] reported the administration of HCF under strict monitoring to maintain the target concentration, which required a multidisciplinary approach. Furthermore, in some countries, including Japan, FXI concentrate is not commercially available or licensed for use.…”

“…Seven hemorrhagic cases have been reported related to the central neural system in FXI deficiency and most cases, except one, were diagnosed after admission. Among these seven cases, there were two cases of subarachnoid hemorrhage (SAH) [7,8], two cases of spinal hemorrhage [6,10], and one case of cerebellar hemorrhage [4] who underwent surgical treatment. Both SAHs were due to ruptured aneurysm; one was treated using the clipping method and another the coiling method, which reportedly led to a second episode of SAH due to aneurysmal revascularization and rupture.…”

“…In general, patients with FXI deficiency are known to have unpredictable bleeding tendencies after surgery [1,2]. To date, only seven hemorrhagic cases related to the central neural system have been reported in FXI deficiency [4][5][6][7][8][9][10]. Herein, we report a case of cerebral hemorrhage in a FXI deficiency patient who was first diagnosed after surgery.…”