Nephrotic syndrome as a complication of chronic graft-versus-host disease after allogeneic haemopoietic stem cell transplantation

• Published in: Internal medicine journal Vol. 46; no. 6; pp. 737 - 741
• Main Authors: Wong, E., Lasica, M., He, S. Z., Bajel, A., Roberts, A. W., Mason, K. D., Ritchie, D. S., Szer, J.
• Format: Journal Article
• Language: English
• Published: Melbourne John Wiley & Sons Australia, Ltd 01.06.2016
• Subjects: Adrenal Cortex Hormones - therapeutic use; Adult; Female; Graft vs Host Disease - complications; Graft vs Host Disease - drug therapy; graft-versus-host disease; Hematopoietic Stem Cell Transplantation - adverse effects; Humans; Immunosuppression - adverse effects; Incidence; Kidney Glomerulus - pathology; Male; Middle Aged; nephrotic syndrome; Nephrotic Syndrome - complications; Nephrotic Syndrome - drug therapy; Postoperative Complications - drug therapy; stem cell transplantation; stem cell transplantation; graft-versus-host disease; nephrotic syndrome
• ISSN: 1444-0903; 1445-5994
• DOI: 10.1111/imj.13098

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.