title: Successful Kidney Transplantation in Epstein Syndrome With Antiplatelet Antibodies and Donor-specific Antibodies: A Case Report.
abstract: An autosomal dominant hereditary disease, Epstein syndrome (ES) is characterized by sensorineural hearing impairment, macrothrombocytopenia, and hereditary nephritis, and can progress to end-stage kidney disease after puberty. Generally, kidney transplantation is difficult to perform in Epstein syndrome owing to the high risk of perioperative bleeding. Additionally, due to previous platelet transfusions, ES patients sometimes have antihuman leukocyte antigen (HLA) antibodies, including antiplatelet antibodies and donor-specific anti-HLA antibodies (DSA), which may result in refractoriness to platelet transfusion and antibody-mediated rejection (AMR). We report a case of successful kidney transplantation in a patient with ES who had DSA and antiplatelet antibodies. To prevent AMR, we used a desensitization protocol (a combination of plasmapheresis, rituximab, and basiliximab induction). Surveillance biopsy performed at 4 months and 1 year after transplantation showed no pathological findings suggesting AMR. To prevent perioperative bleeding complications, we infused the patient with HLA-matched platelets, thereby maintaining the platelet count at >10.0 × 10(4)/μL, and no postoperative episodes of bleeding occurred.
authors.affiliation: Department of Pediatric Nephrology, Faculty of Medicine, Toho University, Tokyo, Japan; Department of Nephrology, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.
authors.forename: J
authors.initials: J
authors.lastname: Hashimoto
authors.affiliation: Department of Pediatric Nephrology, Faculty of Medicine, Toho University, Tokyo, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Hamasaki
authors.affiliation: Department of Nephrology, Faculty of Medicine, Toho University, Tokyo, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Yanagisawa
authors.affiliation: Department of Pediatrics, Toho University School of Medicine Ohashi Hospital, Tokyo, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Sekine
authors.affiliation: Department of Nephrology, Faculty of Medicine, Toho University, Tokyo, Japan.
authors.forename: A
authors.initials: A
authors.lastname: Aikawa
authors.affiliation: Department of Pediatric Nephrology, Faculty of Medicine, Toho University, Tokyo, Japan; Department of Nephrology, Faculty of Medicine, Toho University, Tokyo, Japan. Electronic address: shishido@med.toho-u.ac.jp.
authors.forename: S
authors.initials: S
authors.lastname: Shishido
mesh_terms: D000328:Adult; D001706:Biopsy; D003888:Desensitization, Immunologic / Q000379:methods; D006084:Graft Rejection / Q000517:prevention & control*; D006085:Graft Survival / Q000276:immunology*; D006680:HLA Antigens / Q000276:immunology; D006319:Hearing Loss, Sensorineural / Q000276:immunology / Q000601:surgery*; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D007518:Isoantibodies / Q000276:immunology*; D016030:Kidney Transplantation / Q000379:methods*; D008297:Male; D010956:Plasmapheresis; D000069283:Rituximab / Q000627:therapeutic use; D013921:Thrombocytopenia / Q000151:congenital* / Q000276:immunology / Q000601:surgery; D014019:Tissue Donors
chemical_list: D006680:HLA Antigens; D007155:Immunologic Factors; D007518:Isoantibodies; D000069283:Rituximab