Production and Therapeutic Use of a Factor XI Concentrate from Plasma

Bolton‐Maggs, Paula; Wensley, R. T.; Kernoff, P. B. A.; Kasper, Carol K.; Winkelman, L.; Lane, R. S.; Smith, J. K.

doi:10.1055/s-0038-1648439

Cited by 68 publications

(53 citation statements)

References 7 publications

(9 reference statements)

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“…The concomitant use of tranexamic acid and FXI concentrate should be avoided. The half-life of FXI, as determined in 19 patients, was 52 ± 22 h (mean ± SD) [98], and therefore subsequent doses are seldom needed. Recombinant FVIIa has a half-life of two hours and repeat doses may be required or treatment continued with tranexamic acid.…”

Section: Factor XI Deficiencymentioning

confidence: 98%

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: 98%

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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“…FXI has a relatively long half-life (40-70 hours), so that the infusion of 15 to 20 mL/kg virus-inactivated FFP at alternate days should be sufficient to keep FIX at trough hemostatic levels of 15% to 20% (Table 6). Plasma concentrates of FXI are manufactured in France and in the United Kingdom, 88 but thrombotic complications due to hypercoagulability were observed. [89][90][91] Manufacturers have tried to circumvent this problem by adding heparin and/or protease inhibitors to concentrates (Table 5).…”

Section: Org Frommentioning

confidence: 99%

Recessively inherited coagulation disorders

Mannucci

Duga

Peyvandi

2004

Blood

452

465

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Deficiencies of coagulation factors other than factor VIII and factor IX that cause bleeding disorders are inherited as autosomal recessive traits and are rare, with prevalences in the general population varying between 1 in 500 000 and 1 in 2 million for the homozygous forms. As a consequence of the rarity of these deficiencies, the type and severity of bleeding symptoms, the underlying molecular defects, and the actual management of bleeding episodes are not as well established as for hemophilia A and B. We investigated more than 1000 patients with recessively inherited coagulation disorders from Italy and Iran, a country with a high rate of recessive diseases due to the custom of consanguineous marriages.Based upon this experience, this article reviews the genetic basis, prevalent clinical manifestations, and management of these disorders. The steps and actions necessary to improve the condition of these often neglected patients are out-

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“…All three of our cases received epidural anaesthesia with no adverse outcome, and there are similar reports in the literature 3 . Factor XI concentrate 22 has been used successfully since 1984, although thrombogenic adverse events have been reported with its use 23 . These occur especially in individuals with pre-existing cardiovascular disease and coagulation activation and therefore it should be used with caution in pregnancy and puerperium 20 .…”

Section: Discussionmentioning

confidence: 99%

Factor XI deficiency presenting in pregnancy: diagnosis and management

David

2002

BJOG: An International Journal of Obstetrics and Gynaecology

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A 34 year old primagravidae had an uneventful dichorionic diamniotic twin pregnancy, with normal fetal growth on ultrasound and both twins in a cephalic presentation. Labour was induced at 40 +5 weeks of gestation with vaginal prostaglandin and an epidural catheter was sited. The first twin was delivered by rotational forceps delivery for delay in the second stage of labour secondary to deep transverse arrest, and a right mediolateral episiotomy was made just before delivery. The second twin was delivered 19 minutes later by ventouse extraction for fetal distress using a silicone cup. The third stage of labour was actively managed and the uterus contracted well; however, following delivery of the second twin, profuse vaginal bleeding was noticed from the episiotomy site and two bilateral vaginal tears that extended to one-third of the length of the vagina. No other genital trauma was present and the vaginal lacerations and episiotomy were repaired without delay. The estimated blood loss was 1500 mL. A coagulation screen showed an isolated prolonged activated partial thromboplastin time of 61 seconds (control 34 seconds, normal range 36 -50 seconds), corrected to 48 seconds in vitro by a 50:50 mix with normal plasma. The haemoglobin concentration was 6.5 g/dl and the woman received a transfusion of five units of blood. The activated partial thromboplastin time following transfusion was 49 seconds. She had no history of bleeding following trauma or surgery, which included fractured tibia and fibula, spinal surgery for lumbar vertebral fracture and appendicectomy. There was no family history of a bleeding tendency. Clotting studies later indicated a low level of factor XI of 0.16 U/mL (normal range 0.7 -1.5 U/mL) and normal levels of factors VIII, IX and XII. This confirmed a diagnosis of factor XI deficiency. Lupus anticoagulant and antiphospholipid antibodies were not present. Postpartum recovery of the mother and both babies was uneventful with no neonatal bleeding complications and both infants had normal levels of factor XI. Case 2A 34 year old woman had an uneventful pregnancy. She was admitted with a nine-hour history of spontaneous rupture of the membranes at 39 weeks of gestation. Her previous delivery at another hospital was an emergency lower segment caesarean section for failed induction of labour following spontaneous rupture of membranes at 37 weeks of gestation. This was reported to be complicated by excessive blood loss although coagulation studies were not performed at the time. An appendicectomy was complicated by delayed wound haemorrhage on the second post-operative day. The wound required resuturing. She returned to theatre because of secondary haemorrhage following tonsillectomy and required a blood transfusion. Laser cone biopsy and diagnostic laparoscopy had been uneventful: her sister has bled following a diagnostic laparoscopy.A repeat caesarean section was planned when labour has not begun 24 hours after admission. A coagulation screen performed because of her bleeding history showed an i...

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Production and Therapeutic Use of a Factor XI Concentrate from Plasma

Cited by 68 publications

References 7 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

Recessively inherited coagulation disorders

Factor XI deficiency presenting in pregnancy: diagnosis and management

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