10 Factor XI deficiency

Bolton‐Maggs, Paula

doi:10.1016/s0950-3536(96)80068-0

Cited by 30 publications

(28 citation statements)

References 52 publications

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“…Factor XI (FXI) deficiency is a rare autosomal coagulation disorder with an estimated prevalence of 1 in 1 000 000 individuals [1]. It is the second most commonly reported rare bleeding disorder according to the current global survey of the World Federation of Haemophilia [2].…”

Section: Introductionmentioning

confidence: 99%

“…This disorder is observed in many parts of the world and the incidence of severe form is very population dependent. Unlike haemophilia, the clinical manifestations of this disorder are generally mild [1,3,4]. Most severely FXI-deficient patients (FXI activity below 20 IU dL −1 ) are at a higher risk of bleeding, but some patients shows no manifestations.…”

Section: Introductionmentioning

confidence: 99%

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Factor XI replacement for inherited factor XI deficiency in routine clinical practice: results of the HEMOLEVEN prospective 3‐year postmarketing study

et al. 2015

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Factor XI (FXI)-deficient patients may develop excessive bleeding after trauma or surgery. Replacement therapy should be considered in high-risk situations, especially when FXI levels are below 20 IU dL−1. HEMOLEVEN is a human plasma-derived factor XI concentrate available in France since 1992, but there are few data regarding its use by physicians. This prospective study assessed the use, efficacy and safety of HEMOLEVEN in common clinical practice. HEMOLEVEN was evaluated in FXI-deficient patients in 13 French centres in a 3-year postmarketing study. Forty-four patients (30 females, 14 males) received 67 treatments. The median age was 37 years (8 months–91 years). Basal FXI levels were <1 to 51 IU dL−1 (median: 5.5); 29 patients were severely FXI-deficient (<20 IU dL−1). FXI was administered prophylactically before 43 surgical procedures, 10 invasive procedures, 8 vaginal deliveries, or as curative treatment for six bleeds. The efficacy was assessed as excellent/good in 63, moderate in two and undetermined in two treatments. Seven patients experienced seven adverse effects, including two rated as serious: one sudden massive pulmonary embolism with fatal outcome and one case of inhibitor to FXI. HEMOLEVEN is effective for bleeding prevention in FXI deficiency. However, considering the benefit/risk ratio observed in relation to dosage in this study; firstly, it should be used sparingly due to its potential prothrombotic effect; secondly, new prescription procedures should be defined to adapt the dosage, especially in patients with intrinsic and/or acquired risk factors for thrombosis.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Factor XI replacement for inherited factor XI deficiency in routine clinical practice: results of the HEMOLEVEN prospective 3‐year postmarketing study

et al. 2015

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“…Die Aktivierung des Faktors XI selbst erfolgt durch den Faktor XII, wobei jedoch 1991 erkannt wurde, dass auch Thrombin die Fähigkeit zur Faktor-XI-Aktivierung besitzt. Somit erhält der Gerinnungsfaktor XI in der ¹revidierten Kaskadentheorieª einen neuen Stellenwert [3,16].…”

Section: Introductionunclassified

“…Spontane Blutungen sind nur bei einem Teil der Patienten vorhanden und äuûern sich insbesondere in Schleimhautblutungen (Mund-, Nasen-, Rachenraum, Urogenitalsystem mit Menorrhagien und postpartalen Hämorrhagien). Die Blutungsneigung kann aber auch bei sonst symptomlosen Patienten nach Traumata oder chirurgischen Eingriffen bedeutungsvoll werden [3,12,14,16,20].…”

Section: Introductionunclassified

Postoperative Blutung bei einem Mädchen mit angeborenem Faktor-XI-Mangel - erfolgreiche Therapie mit Desmopressin (DDAVP, Minirin®)

Heim

Lutze²,

Aumann³

et al. 2002

Klin Padiatr

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“…FXI deficiency is an unusual coagulopathy in that its bleeding manifestations vary widely, ranging from a complete absence of symptoms to injury‐related bleeding that appears in areas of increased fibrinolysis [3]. FXI deficiency rarely manifests itself as a spontaneous haemorrhage.…”

Section: Introductionmentioning

confidence: 99%

A case of chronic myelomonocytic leukaemia and factor XI deficiency with a circulating anticoagulant

Billon¹,

Blouch²,

Escoffre‐Barbe³

et al. 2001

Haemophilia

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Inhibitors against factor XI (FXI) have been frequently described in patients who acquired inhibitors (due to auto‐inmune disorders, malignancies or infections), but less often in those with a congenital deficiency of this factor, who had received plasma infusions. The present report concerns one such inhibitor found in the plasma of a patient with chronic myelomonocytic leukaemia and infected by B19 parvovirus, who was neither a heterozygote nor a homozygote for FXI deficiency, and who had no bleeding tendency despite a very low FXI level. Taking this case into account, we discuss and present the clinical and biological features of acquired FXI deficiency caused by an inhibitor.

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10 Factor XI deficiency

Cited by 30 publications

References 52 publications

Factor XI replacement for inherited factor XI deficiency in routine clinical practice: results of the HEMOLEVEN prospective 3‐year postmarketing study

Factor XI replacement for inherited factor XI deficiency in routine clinical practice: results of the HEMOLEVEN prospective 3‐year postmarketing study

Postoperative Blutung bei einem Mädchen mit angeborenem Faktor-XI-Mangel - erfolgreiche Therapie mit Desmopressin (DDAVP, Minirin®)

A case of chronic myelomonocytic leukaemia and factor XI deficiency with a circulating anticoagulant

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