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

Goto, Yukihiro; Taniyama, Ichita; Ebisu, Toshihiko; Mineura, Katsuyoshi

doi:10.1097/mbc.0b013e328353a5e3

Cited by 5 publications

(3 citation statements)

References 14 publications

(27 reference statements)

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“…While neither FXI levels nor aPTT values were found to correlate with bleeding risk among FXI‐deficient adult patients, 22 there was a significant correlation between FXI levels and the presence of bleeding symptoms among our pediatric patients. Three patients in our cohort sustained an ICH, which is a rare complication among patients with FXI deficiency and only scarcely reported 23,24 . Notably, there were no cases of in utero or perinatal ICH among our patients.…”

Section: Discussionmentioning

confidence: 62%

Pediatric severe factor XI deficiency: A multicenter study

Barg

Levy‐Mendelovich

Budnik

et al. 2021

Pediatric Blood & Cancer

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Background Factor XI (FXI) deficiency is a rare autosomal recessive bleeding disorder. Only scarce publications address its clinical features in children. The increased prevalence of FXI deficiency in Israel enabled data collection for this large multicenter cohort study. Objective Some hemostatic challenges may be unique or more common in children, such as bleeding in the neonatal period or trauma‐related injury. The current study was designed to explore the potential impact of these differences in children with severe FXI deficiency. Methods Medical files of all children with FXI level under 15% followed at five tertiary centers were evaluated. The retrieved data comprised demographic and clinical characteristics, including bleeding episodes, surgical interventions, treatment strategies, as well as laboratory features. Results Sixty children, whose median age at diagnosis was 4.2 years and their median FXI level was 4%, were included. Three children experienced triggered intracranial hemorrhage (ICH) and two children had major bleeds. No bleeding complications occurred in surgeries in which hemostatic treatment consisting mostly of tranexamic acid or fresh frozen plasma was applied (n = 45). In contrast, excessive bleeding was noted in 25% of surgical procedures performed without hemostatic preparation (p = .002). Conclusion This study's findings confirm the generally favorable outcome of this rare bleeding disorder, with no spontaneous bleeds or cases of perinatal ICH. Nonetheless, proper diagnosis and adequate hemostasis in the surgical setting are imperative. Unlike previous studies in adults, our pediatric study suggests an association between the severity of FXI deficiency and bleeding tendency.

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Section: Discussionmentioning

confidence: 62%

Pediatric severe factor XI deficiency: A multicenter study

Barg

Levy‐Mendelovich

Budnik

et al. 2021

Pediatric Blood & Cancer

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show abstract

“…However, the increasingly common preoperative coagulation screen testing indicates a greater‐than‐expected incidence . In addition, genetic mutations causing FXI deficiency have been reported in groups of other Middle East, Far East, and white ethnicities . Hence, it is not surprising that slightly more than one‐half of the patients in our series had no known Jewish ancestry, and many cases of FXI deficiency were discovered during preoperative work‐up.…”

Section: Discussionmentioning

confidence: 86%

Therapeutic plasma exchange for perioperative management of patients with congenital factor XI deficiency

Alsammak

Ashrani

Winters

et al. 2017

J of Clinical Apheresis

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“…Neurosurgical intervention in the acute setting of intracranial hemorrhage in patients with HC is often reserved for those with a deteriorating neurological exam and /or/ clinically significant hemorrhage with mass effect. Beyond prompt surgical intervention the perioperative management of HC is of paramount importance to optimize outcomes [2,3]…”

Section: Introductionmentioning

confidence: 99%

A spontaneous cerebral hemorrhage case in the right temporal lobe in a patient with hemophilia C (XI factor deficiency)

Khachatryan¹,

Harutyunyan²,

Nazinyan³

et al. 2023

GSC Adv. Res. Rev.

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A 28-year-old man was taken to our hospital with sudden onset of severe headache. The computed tomography (CT) scan revealed subcortical hemorrhage of the right temporal lobe. The patient has past medical history of hemophilia C (XI factor deficiency). On neurological examination no deficit was observed (Glasgow Coma Scale (GCS) -15). 10 hours after admission, his GCS and CT scan of the brain were unchanged. After consultation with the hematologist, he received fresh frozen plasma, mannitol, tranexamic acid during the treatment. But 36 hours after admission, the neurological status was worsened (GCS -12)․ A decision was made to immediately perform a temporal craniotomy with evacuation of intracerebral hematoma. Surgery and the postoperative period were uneventful․ The patient was discharged without neurological deficit․ This case highlights the importance of adequate preparation of the patient presurgically as much as possible to minimizes the risk of complications (rebleeding) during the operation and in the postoperative period.

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

Cited by 5 publications

References 14 publications

Pediatric severe factor XI deficiency: A multicenter study

Pediatric severe factor XI deficiency: A multicenter study

Therapeutic plasma exchange for perioperative management of patients with congenital factor XI deficiency

A spontaneous cerebral hemorrhage case in the right temporal lobe in a patient with hemophilia C (XI factor deficiency)

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