A case of kidney transplantation for complete C4 deficiency with recurrent IgM-monoclonal gammopathy of renal significance (MGRS) associated nephropathy
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Abstract
We present a rare case of a patient with complete C4 deficiency who underwent kidney transplantation and experienced immunoglobulin M-monoclonalgammopathyofrenalsignificance (IgM-MGRS) recurrence after the procedure. A 45-year-old male patient presented with end-stage renal failure due to membranoproliferative glomerulonephritis (MPGN). The initial immunosuppressive regimen consisted of tacrolimus, steroids, mycophenolate mofetil, basiliximab, and rituximab. He underwent ABO-incompatible kidney transplantation from his mother in August 2021. The clinical course after kidney transplantation was uneventful for a month. A biopsy of the transplanted kidney was performed due to decreased renal function. The allograft biopsy result led to the suspicion of primary macroglobulinemia-associated nephropathy. Bone marrow biopsy revealed an increase in plasma cells; however, no diagnosis of primary macroglobulinemia was made. At this point, IgM-MGRS was diagnosed instead of primary macroglobulinemia. A follow-up allograft biopsy was performed, and IgM-MGRS-associated nephropathy was diagnosed. Eventually, his retrieved autologous kidney biopsy from the initial examination showed that the primary disease was not MPGN but recurrent IgM-MGRS-associated nephropathy. Dexamethasone, rituximab, and cyclophosphamide (DRC) were started to treat IgM-MGRS due to worsening renal function (serum creatinine levels were in the 4–5 mg/dL range). Additional doses of DRC with 20 cycles of plasma exchange were introduced. Severe side effects occurred but did not result in death.
期刊介绍:
To provide to national and regional audiences experiences unique to them or confirming of broader concepts originating in large controlled trials. All aspects of organ, tissue and cell transplantation clinically and experimentally. Transplantation Reports will provide in-depth representation of emerging preclinical, impactful and clinical experiences. -Original basic or clinical science articles that represent initial limited experiences as preliminary reports. -Clinical trials of therapies previously well documented in large trials but now tested in limited, special, ethnic or clinically unique patient populations. -Case studies that confirm prior reports but have occurred in patients displaying unique clinical characteristics such as ethnicities or rarely associated co-morbidities. Transplantation Reports offers these benefits: -Fast and fair peer review -Rapid, article-based publication -Unrivalled visibility and exposure for your research -Immediate, free and permanent access to your paper on Science Direct -Immediately citable using the article DOI