title: Chronic graft-versus-host disease complicated by nephrotic syndrome.
abstract: Chronic graft-versus-host disease (cGVHD) is one of the most frequent and serious complications of allogeneic hematopoietic stem cell transplantation (HSCT). Nephrotic syndrome (NS) is an uncommon and underrecognized manifestation of cGVHD. We report a patient who developed NS 18 months after allogeneic bone marrow transplantation. The onset of NS was accompanied by active manifestations of cGVHD, and immunosuppressants had not been tapered recently. Renal biopsy revealed membranous nephropathy. The patient failed to improve with three combined immunosuppressants (prednisolone, cyclosporine, and mycophenolate mofetil), but achieved partial remission after intravenous immunoglobulin (IVIG) infusion. Twenty-four months after the diagnosis of NS, the patient was still in hematological remission, with normal serum creatinine level, urinary protein loss of 0.7-1.9 g/day and mild oral mucositis. Our report suggests that NS can be a cGVHD-related immune disorder in HSCT patients. Monitoring of renal parameters, especially proteinuria, is important in cGVHD patients. Our case indicated that post-transplant NS, occurring without history of tapering or following immunosuppressant withdrawal, presents a more severe activity of cGVHD and a relatively severe clinical course. IVIG may modify and control the refractory GVHD-related NS, and can be one of the choices of treatment.
authors.affiliation: Department of Pediatrics, Division of Nephrology, Taipei Veterans General Hospital, Taipei, Taiwan, ROC.
authors.forename: Hsin-Hui
authors.initials: HH
authors.lastname: Wang
authors.forename: An-Hang
authors.initials: AH
authors.lastname: Yang
authors.forename: Ling-Yu
authors.initials: LY
authors.lastname: Yang
authors.forename: Giun-Yi
authors.initials: GY
authors.lastname: Hung
authors.forename: Jei-Wen
authors.initials: JW
authors.lastname: Chang
authors.forename: Chun-Kai
authors.initials: CK
authors.lastname: Wang
authors.forename: Tzong-Yann
authors.initials: TY
authors.lastname: Lee
authors.forename: Ren-Bin
authors.initials: RB
authors.lastname: Tang
mesh_terms: D016026:Bone Marrow Transplantation; D002648:Child; D002908:Chronic Disease; D006086:Graft vs Host Disease / Q000150:complications*; D006801:Humans; D007165:Immunosuppression Therapy; D008297:Male; D009404:Nephrotic Syndrome / Q000209:etiology* / Q000628:therapy; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000628:therapy; D014184:Transplantation, Homologous