Transfusion-Induced Specific Anti–Factor XI Inhibitor in a Patient with Previously Unrecognized Factor XI Deficiency

Ce, Musclow; Amato, Dante; Ofosu, Frederick A.; Al, Armstrong; Abbott, David H.

doi:10.1093/ajcp/89.3.418

Cited by 13 publications

(9 citation statements)

References 12 publications

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“…Nearly all patients with congenital deficiency of Factor XI developing anti-Factor XI inhibitors have been previously transfused with plasma or some blood product. 25,[116][117][118][119] Of interest, the hemorrhagic manifestations of these patients do not worsen as opposed to the situation in which a patient having mild hemophilia A or B develops an inhibitor. In one instance, the Factor XI activity decreased from 52 to < 1 U/dl; yet this patient developed no spontaneous hemorrhage.…”

Section: Inhibitors Of Factor XImentioning

confidence: 99%

Factor XI: A Review of Its Biochemistry and Deficiency

Kitchens¹

1991

Semin Thromb Hemost

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The biochemistry and physiology of Factor XI have been reviewed. The clinical history of our 25 patients was reviewed and compared with others' experiences. Factor XI deficiency remains enigmatic in that there is no correlation between Factor XI levels and clinical manifestations. Approximately 40 to 50% of all persons lacking Factor XI are of Ashkenazi Jewish extraction, and the remainder are represented by nearly all populations. Persons may be found to lack Factor XI because of evaluation for hemorrhage, evaluation of a prolonged PTT, or through family or other genetic studies. Hemorrhage is not as severe as in patients with hemophilia A or B. Data are presented suggesting that hemorrhage may, in part, be associated with aspirin use.

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Section: Inhibitors Of Factor XImentioning

confidence: 99%

Factor XI: A Review of Its Biochemistry and Deficiency

Kitchens¹

1991

Semin Thromb Hemost

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“…Patients with severe factor XI deficiency who have received plasma infusions can develop non-physiologic inhibitors to factor XI [14][15][16][17][18]. Patients with factor XI inhibitor can be treated with the factor XI concentrates with or without inhibitors of fibrinolysis.…”

Section: Introductionmentioning

confidence: 99%

Treatment of factor XI inhibitor using recombinant activated factor VIIa

et al. 2005

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A 30-year-old female with severe factor XI deficiency of 0-2% acquired factor XI inhibitor following many infusions for fresh frozen plasma (FFP) for surgical procedures starting at 4 years of age. Seven months before this inhibitor was diagnosed, surgery was complicated by prolonged bleeding resistant to FFP, requiring epsilon aminocaproic acid (EACA) and surgical packing. The inhibitor was measured at 2.2 Bethesda units, 7 months since the last FFP. The inhibitor was confirmed as specific anti-XI and anti-XIa binding by patient's IgG to immobilized factor XI and factor XIa from whole plasma and purified IgG. For repair of a painful anterior cruciate ligament (ACL) defect she was given recombinant factor VIIa (rVIIa) at 90 mug kg(-1), starting one-half hour preoperatively and continued every 2 h for 8 h when haemostasis was complete. Thereafter the rVIIa was given every 3 h for two doses, and then every 4 h for four doses at which time she was discharged on EACA which was continued for 6 days. There was excellent haemostasis during and following the surgery. There was no evidence of consumptive coagulopathy, with no change in the fibrinogen, platelet count, or D-D dimer; and no increase of platelet factor 4, beta-thromboglobulin, or prothrombin fragment F 1.2. The thrombin-antithrombin complex increased over baseline after 24 h. There was no postoperative deep vein thrombosis or pulmonary embolus. In this patient with a factor XI inhibitor, the recombinant factor VIIa was effective and safe, ensuring adequate haemostasis with no thrombotic complications. This product which was designed for patients with inhibitors to factor VIII or factor IX, and factor VII deficiency, has now been given successfully to four patients with factor XI inhibitors.

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“…6 The development of inhibitors to factor XI was reported in anecdotal cases with severe inherited factor XI deficiency following plasma infusion. [7][8][9][10][11][12][13][14][15] Most such patients do not bleed spontaneously but can bleed seriously during or after surgery. The reported inhibitors were shown to bind to factor XI and interfere with factor XI and/or factor IX activation by factor XIa.…”

Section: Introductionmentioning

confidence: 99%

Prevalence, causes, and characterization of factor XI inhibitors in patients with inherited factor XI deficiency

Salomon

Zivelin²,

Livnat³

et al. 2003

Blood

120

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Factor XI deficiency, an injury-related bleeding disorder, is rare worldwide but common in Jews in whom 2 mutations, Glu117Stop (type II) and Phe283Leu (type III), prevail. Mean factor XI activities in homozygotes for Glu117Stop and for Phe283Leu are 1 and 10 U/dL, respectively. Inhibitors to factor XI in patients with severe factor XI deficiency have been reported in a small number of instances. This study was undertaken to determine the prevalence of acquired inhibitors against factor XI in patients with severe factor XI deficiency, discern whether these inhibitors are related to specific mutations, and characterize their activity. Clinical information was obtained from unrelated patients with severe factor XI deficiency, and blood was analyzed for factor XI activity, inhibitor to factor XI, and causative mutations. Immunoglobulin G purified from patients with an inhibitory activity was tested for binding to factor XI, effects on activation of factor XI by factor XIIa and thrombin, and activation of factor IX by exogenous factor XIa. Of 118 Israeli patients, 7 had an inhibitor; all belonged to a subgroup of 21 homozygotes for Glu117Stop who had a history of plasma replacement therapy. Three additional patients with inhibitors from the United Kingdom and the United States also had this genotype and were exposed to plasma. The inhibitors affected factor XI activation by thrombin or factor XIIa, and activation of factor IX by factor XIa.

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Transfusion-Induced Specific Anti–Factor XI Inhibitor in a Patient with Previously Unrecognized Factor XI Deficiency

Cited by 13 publications

References 12 publications

Factor XI: A Review of Its Biochemistry and Deficiency

Factor XI: A Review of Its Biochemistry and Deficiency

Treatment of factor XI inhibitor using recombinant activated factor VIIa

Prevalence, causes, and characterization of factor XI inhibitors in patients with inherited factor XI deficiency

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