Primary amyloidosis has unfavorable prognosis, particularly with organ involvement. Here, we report a case of clinical remission of renal amyloidosis after autologous hematopoietic cell transplantation. A 51-year-old female patient visited our hospital due to generalized edema. Initial evaluation showed hyperlipidemia, hypoalbuminemia, and heavy proteinuria, which were consistent with nephrotic syndrome. However, IgM lamda type monoclonal gammopathy was detected in serum and urine electrophoresis studies. Renal biopsy showed Congo red-positive amyloid deposition in mesangial area, glomerular capillary walls, and arterioles and amyloid fibers were confirmed by electron microscopy. Immunohistochemial study of the biopsy tissue demonstrated systemic light-chain amyloidosis (AL amyloidosis). Multiple myeloma was not evident on bone marrow examination. She received autologous hematopoietic cell transplantation after high dose melphalan treatment. Complete remissions were achieved after the treatment, respectively. Our findings suggest the potential role of autologous peripheral blood stem cell transplantation in treatment of AL amyloidosis.

Nephrotic syndrome is most commonly observed in membranous lupus nephritis in patients with systemic lupus erythematosus (SLE). However, other forms of idiopathic nephrotic syndrome rarely occur in these patients. Here, we report a case of SLE complicated by minimal change disease (MCD). A 24-year-old woman with SLE visited our hospital for generalized edema and heavy proteinuria. Laboratory tests did not support immunological exacerbation of lupus, while renal biopsy revealed diffusely effaced foot processes without electron-dense deposits that were consistent with MCD. Administration of high-dose corticosteroids and 6 subsequent cycles of monthly intravenous cyclophosphamide resulted in complete remission. Although nephrotic-range proteinuria recurred 1 month after switching to maintenance therapy with mycophenolate mofetil, complete remission was reestablished after a 6-month treatment with corticosteroids and cyclosporine. Physicians should be cautious in assessment and management of such a rare renal manifestation.

Citations

Citations to this article as recorded by  

• A Unique Cause of Proteinuria in Pregnancy: Class II Lupus Nephritis with Concomitant Minimal Change Disease
  Ryan Kunjal, Rabie Adam-Eldien, Raafat Makary, Francois Jo-Hoy, Charles W. Heilig
  Case Reports in Nephrology and Dialysis.2016; 6(3): 101.     CrossRef