Management of factor XI inhibitor for cardiac intervention: successful treatment with immunosuppressive therapy and plasma exchange

Teruya, Jun; Styler, Michael

doi:10.1046/j.1365-2516.2000.00385.x

Cited by 22 publications

(28 citation statements)

References 8 publications

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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

122

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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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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

122

View full text Add to dashboard Cite

show abstract

“…Though cardiac interventions and other bypass surgery in patients with factor XI deficiency have been reported [15,16,17], the development of inhibitor to factor XI in congenital deficient patient poses a significant problem and the literature regarding the management of such patient is limited [18]. Two cases of cardiac intervention in patients with factor XI inhibitor have been reported with successful treatment with immunosuppressive therapy and plasma exchange.…”

Section: Discussionmentioning

confidence: 99%

Percutaneous Coronary Intervention in a Patient with Congenital Factor XI Deficiency and Acquired Inhibitor

Mungee¹,

Lapin²,

Çavuşoğlu³

et al. 2006

Cardiology

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Background: Factor XI deficiency has been associated with bleeding diathesis mostly secondary to trauma and post-operatively depending on the severity of deficiency. Cases with factor XI deficiency having undergone cardiac surgery and coronary intervention after appropriate replacement therapy have been reported in the past. The presence of inhibitor in factor XI deficiency poses a hematological challenge and literature regarding coronary intervention in such patients is limited. Immunosuppressive therapy, plasma exchange and factor VII product transfusions have been used prior to cardiac interventions in few such reported cases. Method: We report our approach in such a case of Percutaneous Transluminal Coronary Angioplasty in a 72-year-old male of Jewish origin who has congenital factor XI deficiency complicated with acquired inhibitor. Results: In some cases, the acuity of the coronary syndrome may mandate immediate coronary intervention. However, patient’s history of factor XI deficiency and acquired inhibitor pose a major dilemma of further course of action. We performed percutaneous balloon angioplasty in this case with no anti-coagulant and with favorable outcome. Conclusion: Under these circumstances of significant coagulation disorder and based on the case report, we recommend that balloon angioplasty be undertaken with no additional anti-coagulation other than Aspirin.

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“…In one patient, TPE cycles were continued intraoperatively and postoperatively . There no reported complications in the postoperative period and none were diagnosed with exacerbation of hemolysis …”

Section: Review Of the Literaturementioning

confidence: 99%

“…Preoperatively TPE with FFP replacement could also be a useful tool in patients with bleeding disorders when factor concentrates are not widely available or treatment is extremely costly, as in the case of infusion with factor XI concentrates, or could be suggested in patients with inhibitors. There is one reported case of a patient with factor XI deficiency who preoperatively had two TPE cycles with FFP replacement with no bleeding complications and a patient with factor XI deficiency and the presence of inhibitor who had five TPE cycles with FFP replacement preoperatively, however aortic valve replacement resulted in blood clots and cardiac tamponade requiring reoperation …”

Section: Review Of the Literaturementioning

confidence: 99%

Literature review of apheresis procedures performed perioperatively in cardiac surgery for ASFA category indications

Kanellopoulou

Kostelidou

2018

J of Clinical Apheresis

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Background Perioperative therapeutic plasma exchange in patients with cardiovascular diseases poses several challenges, including alterations in intravascular volume, serum electrolytes, the coagulation cascade, and drug pharmacokinetics. Methods This review article summarizes different indications of plasma exchange for patients requiring cardiac surgery based on reported case reports and case series. Results The most common reported indication is plasma exchange for the management of allosensitized cardiac transplant candidate patients in combination with immunosuppressive regimens, which increases the likelihood of obtaining a cross‐match‐negative allograft, improving post‐transplant clinical outcome. The second most common indication is for patients with a history of heparin‐induced thrombocytopenia syndrome that permits the use of heparin in cardiopulmonary bypass in an urgent cardiac surgery. Less common indications are restoration of clotting factors for patients with congenital bleeding disorders or removal of cold agglutinins. No severe complications were described in reported cases. Conclusion Therapeutic plasma exchange is an effective and safe procedure that could be performed preoperatively and intraoperatively in urgent cardiac surgery for the management of antibody‐mediated disorders including allosensitized cardiac transplant candidate patients or patients with heparin‐induced thrombocytopenia syndrome.

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Management of factor XI inhibitor for cardiac intervention: successful treatment with immunosuppressive therapy and plasma exchange

Cited by 22 publications

References 8 publications

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

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

Percutaneous Coronary Intervention in a Patient with Congenital Factor XI Deficiency and Acquired Inhibitor

Literature review of apheresis procedures performed perioperatively in cardiac surgery for ASFA category indications

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