title: Nephrotic syndrome as a complication of chronic graft-versus-host disease after allogeneic haemopoietic stem cell transplantation.
abstract: Nephrotic syndrome (NS) is a rare complication following allogeneic haemopoietic stem cell transplantation (allo-HSCT), with limited current understanding of its pathogenesis. Here, we describe four cases of NS following allo-HSCT diagnosed at our institutions to identify key clinical and pathological features. In addition, a PubMed search was performed to identify existing reports that were pooled together with our cases for analysis. NS occurred as a late complication following allo-HSCT, with median onset 19.5 months after transplant (range: 3.9-84 months). The most common histopathology observed was membranous nephropathy; however, cases of minimal change disease have also been reported. There is a high incidence of prior extra-renal graft-versus-host disease (GvHD), with all four of our cases and 82% of published cases having prior GvHD. Glucocorticosteroids are the most common treatment, with variable degrees of response. Responses to immunosuppression with calcineurin inhibitors and rituximab have been described in steroid-refractory cases.
authors.affiliation: Clinical Haematology and Bone Marrow Transplant Unit, Royal Melbourne Hospital, Melbourne, Victoria, Australia.
authors.forename: E
authors.initials: E
authors.lastname: Wong
authors.affiliation: Clinical Haematology, Austin Health, Melbourne, Victoria, Australia.
authors.forename: M
authors.initials: M
authors.lastname: Lasica
authors.affiliation: Clinical Haematology and Bone Marrow Transplant Unit, Royal Melbourne Hospital, Melbourne, Victoria, Australia.|Clinical Haematology, Austin Health, Melbourne, Victoria, Australia.
authors.forename: S Z
authors.initials: SZ
authors.lastname: He
authors.affiliation: Clinical Haematology and Bone Marrow Transplant Unit, Royal Melbourne Hospital, Melbourne, Victoria, Australia.
authors.forename: A
authors.initials: A
authors.lastname: Bajel
authors.affiliation: Clinical Haematology and Bone Marrow Transplant Unit, Royal Melbourne Hospital, Melbourne, Victoria, Australia.|Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne, Melbourne, Victoria, Australia.
authors.forename: A W
authors.initials: AW
authors.lastname: Roberts
authors.affiliation: Clinical Haematology and Bone Marrow Transplant Unit, Royal Melbourne Hospital, Melbourne, Victoria, Australia.|Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne, Melbourne, Victoria, Australia.
authors.forename: K D
authors.initials: KD
authors.lastname: Mason
authors.affiliation: Clinical Haematology and Bone Marrow Transplant Unit, Royal Melbourne Hospital, Melbourne, Victoria, Australia.|Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne, Melbourne, Victoria, Australia.
authors.forename: D S
authors.initials: DS
authors.lastname: Ritchie
authors.affiliation: Clinical Haematology and Bone Marrow Transplant Unit, Royal Melbourne Hospital, Melbourne, Victoria, Australia.|Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne, Melbourne, Victoria, Australia.
authors.forename: J
authors.initials: J
authors.lastname: Szer
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000328:Adult; D005260:Female; D006086:Graft vs Host Disease / Q000150:complications* / Q000188:drug therapy; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D007165:Immunosuppression Therapy / Q000009:adverse effects*; D015994:Incidence; D007678:Kidney Glomerulus / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000150:complications* / Q000188:drug therapy; D011183:Postoperative Complications / Q000188:drug therapy
chemical_list: D000305:Adrenal Cortex Hormones
keywords: graft-versus-host disease; nephrotic syndrome; stem cell transplantation