Abstract
MYH9-related disease is an autosomal dominant genetic disease characterized by congenital macrothrombocytopenia, sensorineural hearing loss, and progressive kidney failure. Severe cases require kidney replacement therapy in the patient's second decade of life, and thrombocytopenia constitutes a major risk factor for hemorrhagic complications during dialysis initiation or kidney transplantation. Serious bleeding complications have been reported to occur in the perioperative period despite adequate platelet transfusions. Additionally, platelet transfusion may induce the production of anti-platelet antibodies and donor-specific anti-human leukocyte antigen antibodies. We report a case of a 19-year-old woman with MYH9-related disease due to an R702C missense variant in the motor domain of the MYH9 gene who used prophylactic eltrombopag, a thrombopoietin receptor agonist, from 6 weeks before kidney transplantation. Before transplantation, her platelet count increased from 10 × 10(3)/μL to 156 × 10(3)/μL. No major bleeding complications occurred after living donor kidney transplantation. Although perioperative eltrombopag use has been reported in other surgeries, this is the first successful case of living donor kidney transplantation in which perioperative eltrombopag administration allowed platelet transfusions to be avoided. Eltrombopag may serve as a valuable alternative in kidney transplant recipients with MYH9-related disease by avoiding platelet transfusion and reducing the risk of severe bleeding complications.