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Light chain deposition disease (LCDD) is a systemic disorder characterized by deposition of non-amyloid monoclonal light chains. Renal dysfunction is a ubiquitous manifestation of the LCDD disease. Reports suggest that high-dose chemotherapy and autologous stem cell transplantation (ASCT) may be beneficial in the treatment of LCDD. However, the impact of ASCT on renal function is unclear. This study retrospectively reviewed the effect of ASCT on renal function in patients with LCDD.Six patients with LCDD have been transplanted at our institution since 2001. Patients received dexamethasone alone, dexamethasone plus thalidomide or no chemotherapy prior to conditioning. All the patients underwent high-dose melphalan conditioning after stem cell mobilization.Three of the six patients had concurrent multiple myeloma (MM), and one patient was on haemodialysis prior to transplantation. Four patients were male and two were female. The median age was 43.5 years with a median serum creatinine of 2.4 mg/dl and a median estimated glomerular filtration rate (eGFR) of 26.5 ml/min/1.73 m2. Five patients survived ASCT and one died on Day 26 of transplantation. Median follow-up was 31.7 months (range 31.3–60.7 months) after ASCT. Of the surviving patients, all the five achieved a haematological response post-transplantation although two ultimately relapsed and required further chemotherapy. The eGFR of one patient declined with relapse and improved with treatment, while the eGFR of the second patient remained stable throughout relapse and treatment. The patient on haemodialysis prior to transplantation continued to require it afterward, but ultimately received a renal transplant. Median reduction in proteinuria was 92% and median improvement in eGFR was 95%. Of the four evaluable patients all achieved criteria for a renal response after ASCT.ASCT may be an effective therapy for renal dysfunction associated with LCDD. In cases where kidney dysfunction persists after ASCT, a haematological response may permit successful kidney transplantation with improved graft viability and decreased risk of recurrence.