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

2017 ◽  
Vol 32 (6) ◽  
pp. 429-436 ◽  
Author(s):  
Mohamed S. Alsammak ◽  
Aneel A. Ashrani ◽  
Jeffrey L. Winters ◽  
Rajiv K. Pruthi
Keyword(s):  
Plasma Exchange ◽  
Perioperative Management ◽  
Therapeutic Plasma Exchange ◽  
Factor Xi ◽  
Factor Xi Deficiency ◽  
Congenital Factor

Acquired factor XI deficiency and therapeutic plasma exchange

2017 ◽  
Vol 33 (3) ◽  
pp. 427-430 ◽  
Author(s):  
Geoffrey D. Wool ◽  
Angela Treml ◽  
Jonathan L. Miller
Keyword(s):  
Plasma Exchange ◽  
Therapeutic Plasma Exchange ◽  
Factor Xi ◽  
Factor Xi Deficiency

Gynaecological and obstetrical bleeding in Caucasian women with congenital factor XI deficiency: Results from a twenty-year, retrospective, observational study

2019 ◽  
Vol 153 (10) ◽  
pp. 373-379
Author(s):  
Carlos Bravo-Perez ◽  
Teresa Ródenas ◽  
Julio Esteban ◽  
Maria Eugenia de la Morena-Barrio ◽  
Salam Salloum-Asfar ◽  
...  
Keyword(s):  
Observational Study ◽  
Retrospective Observational Study ◽  
Factor Xi ◽  
Factor Xi Deficiency ◽  
Caucasian Women ◽  
Congenital Factor

Perioperative management of factor XI deficiency in a patient undergoing hip arthroplasty

2013 ◽  
Vol 28 (4) ◽  
pp. 618-620 ◽  
Author(s):  
Yohei Yamada ◽  
Kayoko Fujimoto-Ibusuki ◽  
Keiko Morikawa-Kubota
Keyword(s):  
Hip Arthroplasty ◽  
Perioperative Management ◽  
Factor Xi ◽  
Factor Xi Deficiency

Congenital Factor XI Deficiency

2018 ◽  
pp. 291-306
Author(s):  
Tahere Tabatabaei ◽  
Akbar Dorgalaleh
Keyword(s):  
Factor Xi ◽  
Factor Xi Deficiency ◽  
Congenital Factor

Gynaecological and obstetrical bleeding in Caucasian women with congenital factor XI deficiency: Results from a twenty-year, retrospective, observational study

2019 ◽  
Vol 153 (10) ◽  
pp. 373-379
Author(s):  
Carlos Bravo-Perez ◽  
Teresa Ródenas ◽  
Julio Esteban ◽  
Maria Eugenia de la Morena-Barrio ◽  
Salam Salloum-Asfar ◽  
...  
Keyword(s):  
Observational Study ◽  
Retrospective Observational Study ◽  
Factor Xi ◽  
Factor Xi Deficiency ◽  
Caucasian Women ◽  
Congenital Factor

The Use of Factor XI Concentrates (Hemoleven, LFB) in Patients with Congenital Factor XI Deficiency and a Known Bleeding Tendency.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 1796-1796
Author(s):  
Vickie McDonald ◽  
Savidge F. Geoffrey ◽  
Savita Rangarajan ◽  
Mike Mitchell
Keyword(s):  
Fresh Frozen Plasma ◽  
Incidence Rates ◽  
Treatment Modalities ◽  
Bleeding Tendency ◽  
Normal Range ◽  
Factor Xi ◽  
Factor Xi Deficiency ◽  
Unit Dose ◽  
The Mean ◽  
Congenital Factor

Abstract Traditional treatment modalities for FXI deficiency (UK prevalence 400 cases) include antifibrinolytics, desmopressin, fresh frozen plasma (FFP) and FXI concentrates but there has been reluctance to use FXI concentrates because of reported incidence rates of thrombosis up to 10%. Concerns over the safety and efficacy of FFP, with additional viral inactivation steps possibly leading to reduced FXI recoveries, have led us to increase our use of FXI concentrates. We aimed to assess the indications, dosage, recovery, efficacy and safety of Hemoleven, a plasma derived, purified and virally inactivated FXI concentrate, which also contains heparin and antithrombin, in patients with congenital factor XI deficiency. A retrospective study was performed using hospital notes and laboratory records of all patients who had received Hemoleven over a 2-year period. Eleven patients (6 male, 5 female) had been treated with a median age of 38 years (range 7–74) and mean baseline FXI:C levels of 25.4U/dl (3–50). All patients received Hemoleven as prophylaxis for surgery or dental work and had all previously had excess bleeding when surgically challenged. One patient died of a condition unrelated to FXI treatment. Pre- and post-FXI:C levels were available for a total of 60 treatment episodes of which 25 were 1000-unit doses and 35 were 2000-unit doses. The mean increase in FXI:C per 1000-unit dose was 25.4 U/dl (12.4–43.9) while the mean increase in FXI:C per 2000-unit dose was 50.5 U/dl (11.8–106.5). This is consistent with the manufacturer’s data. Ten minute post infusion FXI:C levels were above the normal range (73–133 U/kg) in 8% of patients given 1000 units and 11% of patients given 2000 units but below the normal range in 24% of patients who received 1000 units and 20% of patients who received 2000 units. 90% of treatment episodes led to FXI:C levels above the usual treatment target of 65 U/dl. Genetic analysis of 9/11 patients showed that 2 were homozygous (one type II and one type III), 6 were heterozygous for other recognised mutations and one had no mutation identified but apparent absence of RNA from one allele demonstrated in a relative by qRT-PCR. No excess bleeding or inhibitor development was recorded even in one patient who had had a poor haemostatic response with FFP. There were no episodes of arterial or venous thrombotic complications within this group and no clinical or laboratory evidence of DIC following treatment. In summary, treatment with factor XI concentrates gave consistent increments in FXI:C at the doses given and achieved good haemostasis with no episodes of thrombosis in this study, even in patients over the age of 60y. While the risk of prion transmission is still unknown, use of FXI concentrates is not associated with the risks of fluid overload and TRALI that are seen with FFP. We acknowledge that the study includes small numbers of patients however the cohort of patients with a bleeding diathesis in this condition is small. We conclude that Hemoleven appears to be an effective and reliable treatment for patients with FXI:C deficiency but should be given in the context of FXI:C level monitoring in order to detect those patients who may develop high levels and possible thrombosis.

Successful perioperative management of factor XI deficiency with administration of fresh-frozen plasma in a subdural hematoma patient

2016 ◽  
Vol 16 (1) ◽  
pp. 143-144
Author(s):  
Yoko Edahiro ◽  
Kunimoto Ichikawa ◽  
Hiroharu Suzuki ◽  
Hajime Yasuda ◽  
Michiaki Koike ◽  
...  
Keyword(s):  
Subdural Hematoma ◽  
Perioperative Management ◽  
Fresh Frozen Plasma ◽  
Factor Xi ◽  
Factor Xi Deficiency ◽  
Fresh Frozen ◽  
Frozen Plasma

Successful childbirth by a patient with congenital factor XI deficiency and an acquired inhibitor

1993 ◽  
Vol 84 (1) ◽  
pp. 172-174 ◽  
Author(s):  
Steven S. Ginsberg ◽  
Lionel P. Clyne ◽  
Peter McPhedran ◽  
Thomas P. Duffy ◽  
T. Hanson†
Keyword(s):  
Factor Xi ◽  
Factor Xi Deficiency ◽  
Congenital Factor
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