Factor XI deficiency presenting in pregnancy: diagnosis and management

David, Anna L.; Paterson‐Brown, Sara; Letsky, Elizabeth A.

doi:10.1111/j.1471-0528.2002.00172.x

Cited by 19 publications

(8 citation statements)

References 22 publications

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“…Epidural anaesthesia has been carried out without complication in FXI-deficient patients [54,97]; however, the consequences of a spinal haematoma with compression of the spinal cord makes it an unacceptable risk and epidural should be avoided in severe cases or women with significant bleeding history. If epidural is necessary, it should be covered with FXI concentrate.…”

Section: Factor XI Deficiencymentioning

confidence: 99%

The obstetric and gynaecological management of women with inherited bleeding disorders – review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors’ Organization

et al. 2006

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Summary. The gynaecological and obstetric management of women with inherited coagulation disorders requires close collaboration between obstetrician/ gynaecologists and haematologists. Ideally these women should be managed in a joint disciplinary clinic where expertise and facilities are available to provide comprehensive assessment of the bleeding disorder and a combined plan of management. The haematologist should arrange and interpret laboratory tests and make provision for appropriate replacement therapy. These guidelines have been provided for healthcare professionals for information and guidance and it is also intended that they are readily available for women with bleeding disorders.

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Section: Factor XI Deficiencymentioning

confidence: 99%

The obstetric and gynaecological management of women with inherited bleeding disorders – review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors’ Organization

et al. 2006

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“…Factor XI plays an integral role in the intrinsic pathway of the coagulation cascade (Figure 1) [7]. Initial screening tests for FXI deficiency would reveal an isolated prolonged activated partial thromboplastin time (aPTT).…”

Section: Discussionmentioning

confidence: 99%

“…In a case report by David et al three patients had peripartum epidurals placed (two for Cesarean section, one for labor analgesia), all without adverse outcome. This group recommends coagulation status is restored to normal prior to placing or removing an epidural catheter in the setting of FXI deficiency [7]. Additionally, a case series by Kadir et al described two patients that had epidural anesthesia with no complications.…”

Section: Discussionmentioning

confidence: 99%

Perioperative Management of Patient with Factor XI Deficiency Undergoing Total Knee Arthroplasty

Kamenetsky¹,

Afifi²

2014

J Anesth Clin Res

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Factor XI (FXI) deficiency is a rare, inherited bleeding disorder that is typically asymptomatic and presents with unexpected bleeding after surgery or trauma. We present a patient with FXI deficiency that underwent total knee arthroplasty (TKA) under general anesthesia and received peripheral nerve blocks for postoperative analgesia. We also present a review of literature focused on presentation of FXI deficiency in various surgical settings and choices of perioperative anesthetic management. J o u rn al of A n e s th es ia & C li n ic a l Resea rc h

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“…Tooth extractions can be managed only by tranexamic acid without plasma replacement therapy and skin biopsy can be treated similarly [22]. Epidural anaesthesia is not recommended in patients with severe FXI deficiency without replacement therapy although anecdotal cases have been reported [23].…”

Section: Treatmentmentioning

confidence: 99%

New observations on factor XI deficiency

Salomon

Seligsohn

2004

Haemophilia

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Factor (F) XI is an injury-related bleeding tendency that commonly occurs when trauma involves tissues rich in fibronolytic activators. Severe FXI deficiency is defined when the activity of FXI in plasma is less than 15 U dL(-1). The disorder is inherited as an autosomal recessive trait manifesting in homozygotes or compound heterozygotes, and infrequently in heterozygotes. So far 53 mutations in the gene of FXI have been described and four of them were found to be prevalent in Ashkenazi Jews, Iraqi Jews, Basques or the English population. For each of the four mutations a founder effect was discerned. Inhibitors can develop in patients with FXI level < 1U dL(-1) who were exposed to plasma which seriously complicates their management during surgery. No correction of a prolonged aPTT by normal plasma is indicative of the presence of an inhibitor. In contrast to patients with haemophilia A, severe FXI deficiency provides no protection against myocardial infarction. In patients with severe FXI deficiency undergoing surgery, fresh frozen plasma is the treatment of choice. FXI concentrates can also be used but cause thrombosis in approximately 10% of patients, particularly those with cardiovascular disease. Recombinant FVIIa has successfully prevented bleeding during or after surgery in patients with FXI inhibitors.

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Factor XI deficiency presenting in pregnancy: diagnosis and management

Cited by 19 publications

References 22 publications

The obstetric and gynaecological management of women with inherited bleeding disorders – review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors’ Organization

The obstetric and gynaecological management of women with inherited bleeding disorders – review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors’ Organization

Perioperative Management of Patient with Factor XI Deficiency Undergoing Total Knee Arthroplasty

New observations on factor XI deficiency

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