Goodpasture Syndrome (GS) is an autoimmune disease caused by the development of auto-antibodies against the Glomerular Basement Membrane (GBM). Linear deposit of immunoglobulins G on the GBM detected by immunofluorescence analysis of renal biopsies is a GS pathognomonic finding. GS is commonly monophasic and its incidence is 1.6 case per million per year. This report describes and discusses the case of a 40-year-old woman who one year after allograft kidney transplant, presented with acute pulmonary and renal symptoms of GS, leading to acute graft dysfunction, without circulating anti-GBM antibody detection in laboratory assays. She received a living donor kidney transplant 4 years after the first diagnosis of GS without circulating anti-GBM antibodies, when considered in remission. In both episodes, the diagnosis of GS was based exclusively on the kidney biopsy that showed rapidly progressing glomerulonephritis with deposition of immunoglobulins G on the GBM. Although rare, the management of patients with GS without circulating anti-GBM antibodies is difficult due to the lack of standardized follow-up guidelines to reduce the risk of GS recurrence after kidney transplantation.
Post-transplantation lymphoproliferative disorder (PTLD) is a well-know complication after organ transplantation. We report a case of a patient who developed an extramedullary plasmacytoma-like PTLD around his transplanted kidney treated with standard multiple myeloma chemotherapy. Three years after benefiting of a deceased donor kidney transplant for an end stage kidney disease secondary to nephroangiosclerosis, our patient developed an extra-medullary plasmacytoma confined to the transplant compartment. The transplant function was unaltered, and due to the absence of reduction of the lesion after immunosuppression reduction, a chemotherapy by bortezomib-cyclophosphamide-dexamethasone (VCD) known to be efficient in multiple myeloma was initiated. After 6 cycles, positron emission tomography (PET) scan showed complete metabolic remission confirming the biological exams. This case report suggests that a chemotherapy such as VCD can efficiently treat plasmacytoma-like PTLD allowing graft survival. Therefore, transplant removal may not be mandatory as the best second line treatment after unsuccessfulness reduction of immunosuppression.