Characteristics and posttransplant outcomes of patients with congenital and acquired von Willebrand disease and hemophilia A and with renal transplants.

BACKGROUND

Bleeding disorders such as hemophilia and von Willebrand disease (VWD) have historically been associated with significant morbidity due to hemarthrosis, surgical bleeding, and transfusion requirements. With advances in hemostatic therapy, surgical outcomes have improved; however, data on renal transplantation in this population remain limited.

OBJECTIVES

To assess clinical characteristics and adverse events in renal transplant patients with bleeding diathesis, focusing on primary endpoints: post-transplant bleeding, thrombotic events, and mortality. Secondary endpoints include readmissions, OR takebacks, and renal transplant rejections, including acute cellular rejection and antibody-mediated rejection.

METHODS

A retrospective chart review of renal transplant patients with bleeding diathesis across Mayo Clinic.

RESULTS

The cohort included 11 patients: hemophilia A (3/11) and VWD (8/11), with a mean Kidney Donor Profile Index of 37. Among patients with hemophilia A, 2 had congenital disease, and 1 had previously developed a factor VIII inhibitor that had resolved prior to transplantation. Among VWD patients, 6 had acquired VWD and 2 had type 1 VWD. No patients experienced arterial or venous thrombosis within 1 year. Beyond 1 year, 5 thrombotic events occurred in 4 patients (36.4%): 2 myocardial infarctions, 1 ischemic stroke, and 2 deep vein thromboses; no pulmonary emboli occurred. In the immediate postoperative period (days 0-30), 5 patients (45.5%) had bleeding events-4 major (80%) and 1 minor (20%). No bleeding occurred between days 30 and 365. After 1 year, 4 patients (36.4%) had nonallograft-related bleeding, primarily gastrointestinal. Readmission rates were 36.4% (0-30 days), 27.3% (30-90 days), and 9.1% (90-365 days). Surgical reintervention was required in 18.2% of patients. Rejection occurred in 3 patients (27.3%): 2 with acute cellular rejection, 1 with chronic cellular rejection, and 1 with antibody-mediated rejection. Overall mortality was 45%.

CONCLUSION

Kidney transplant recipients with hemophilia and VWD are at significant perioperative bleeding risk, particularly from perinephric hematomas and intraoperative hemorrhage. The risk decreases after 30 days, but long-term monitoring for both bleeding and thrombosis remains crucial. Rejection rates appear comparable with those of the general transplant population.