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5/6th Nephrectomy in Combination with High Salt Diet and Nitric Oxide Synthase Inhibition to Induce Chronic Kidney Disease in the Lewis Rat
Published on: July 3, 2013
Chronic graft-versus-host disease complicated by nephrotic syndrome
Hsin-Hui Wang1, An-Hang Yang, Ling-Yu Yang
1Department of Pediatrics, Division of Nephrology, Taipei Veterans General Hospital, Taipei, Taiwan, ROC.
Insights
Nephrotic syndrome (NS) is a rare complication of allogeneic stem cell transplants. This case study highlights NS as a potential cGVHD manifestation, suggesting IVIG as a treatment for refractory cases.
Area of Science:
- Nephrology
- Immunology
- Hematology
Background:
- Allogeneic hematopoietic stem cell transplantation (HSCT) can lead to chronic graft-versus-host disease (cGVHD).
- Nephrotic syndrome (NS) is an infrequent but serious complication of cGVHD.
- Early recognition and monitoring of renal function are crucial in HSCT recipients.
Observation:
- A patient developed NS 18 months post-HSCT, concurrent with active cGVHD.
- Renal biopsy confirmed membranous nephropathy.
- Standard immunosuppressants were ineffective, but IVIG induced partial remission.
Findings:
- NS can manifest as a cGVHD-related immune disorder.
- Refractory NS in HSCT patients may indicate severe cGVHD activity.
- Intravenous immunoglobulin (IVIG) showed efficacy in managing refractory cGVHD-related NS.
Implications:
- Routine renal parameter monitoring, especially proteinuria, is vital for cGVHD patients.
- IVIG represents a potential therapeutic option for refractory cGVHD-related NS.
- Understanding NS as a cGVHD complication improves patient management and outcomes post-HSCT.
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.
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