Dataset Information

Inhibitors in hemophilia: association with surgery plans and outcomes in a retrospective cohort study.

ABSTRACT:

Background

The development of antibodies (inhibitors) to clotting factors compromises the management of hemophilia A and B, resulting in resistance to clotting factor replacement and, in many cases, the need for bypassing agents to achieve hemostasis.

Objectives

To evaluate the association between the presence of inhibitors and achievement of perioperative hemostasis, development of complications, and presurgical plan deviations.

Methods

We conducted a retrospective study using data from the Indiana Hemophilia and Thrombosis Center surgical database (1998-2019). Associations between perioperative outcomes and inhibitor status were assessed while controlling for patient and procedural characteristics.

Results

A total of 1492 surgeries were performed in 539 persons with hemophilia, with 72 procedures performed in 20 patients with inhibitors (15 with hemophilia A; 5 with hemophilia B). High-responding inhibitors (>5 BU/mL) were present in 27 procedures, low-responding inhibitors (≤5 BU/mL) were present in in 13 procedures, and 32 procedures were performed in patients with historically persistent inhibitors. Adjusting for age, diagnosis, surgery setting, hemostatic agent, data collection period, and surgery type (major/minor), inhibitors were associated with a higher risk of inadequate perioperative hemostasis (33.4% vs 8.6%; adjusted relative risk [adjRR], 3.78; 95% CI, 1.89-7.56; P < .001). Reported complications include hemorrhage, fever, pain, thrombosis, and infections. Complications were not statistically different based on inhibitor status (31.7% vs 14.6%; adjRR, 1.25; 95% CI, 0.63-2.49; P = .526). Presurgical plan deviations (eg, hemostatic medication dose adjustments, procedure rescheduling, and changes in the length of postoperative hospitalization) occurred more frequently in surgeries involving inhibitors (70.8 vs 39.5%; adjRR, 1.47; 95% CI, 1.12-1.93; P = .005).

Conclusion

Inhibitors are associated with higher risks of adverse perioperative outcomes. Strategies to address inhibitor development should be prioritized to avoid undesirable perioperative outcomes.

SUBMITTER: Olasupo OO

PROVIDER: S-EPMC10704510 | biostudies-literature | 2023 Oct

REPOSITORIES: biostudies-literature

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Publications

Inhibitors in hemophilia: association with surgery plans and outcomes in a retrospective cohort study.

Olasupo Omotola O OO Nakar Charles C Haddix Craig C Matthew Thushara T Matino Davide D Malec Lynn L Mbuagbaw Lawrence L Tarride Jean-Eric JE Iorio Alfonso A Shapiro Amy D AD

Research and practice in thrombosis and haemostasis 20231014 7

<h4>Background</h4>The development of antibodies (inhibitors) to clotting factors compromises the management of hemophilia A and B, resulting in resistance to clotting factor replacement and, in many cases, the need for bypassing agents to achieve hemostasis.<h4>Objectives</h4>To evaluate the association between the presence of inhibitors and achievement of perioperative hemostasis, development of complications, and presurgical plan deviations.<h4>Methods</h4>We conducted a retrospective study u ...[more]

PMID: 38077822

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Dataset Information

Inhibitors in hemophilia: association with surgery plans and outcomes in a retrospective cohort study.

Background

Objectives

Methods

Results

Conclusion

Publications

Inhibitors in hemophilia: association with surgery plans and outcomes in a retrospective cohort study.

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