Light Chain Deposition Disease (LCDD) is a type of monoclonal immunoglobulin deposition disease characterized by the non-amyloid deposition of monoclonal light chains in the tubular basement membranes and Bowman’s capsule. It was first described about 3 decades ago, but due to varied clinical presentations, many differential diagnoses and low incidence, it is both underrecognized and underreported. We present a case of 85-year-old female with past medical history significant for CKD and HTN, who presented with accelerated HTN, normocytic anemia and worsening renal function. Laboratory data showed Hgb <9.5 gm/dL, MCV 93 fL, Total protein 5.9, Albumin 3.2, Calcium 8.9, Serum Creatinine 2.37, BUN 45, Urine with hematuria (50–99 erythrocytes per high-power field) and nephrotic range proteinuria. Renal biopsy showed evidence of Membranoproliferative glomerulonephritis, with immunofluorescence features indicative of a monoclonal immunoglobulin deposition disease. Bone marrow biopsy showed mildly increased plasma cells (5-7%) confirmed to be clonal (lambda light chain) by flow cytometry, negative for Congo-Red stain. Although no underlying hematological abnormality like Multiple Myeloma or Amyloidosis was observed in this case, the renal pathological findings is consistent with proliferative glomerulonephritis with monoclonal IgG lambda deposits. There is no standard of care for the management of LCDD based on rarity of this condition. Many treatment modalities including chemotherapy and stem cell transplant have been tried. A combination of high dose melphalan or Cyclophosphamide with dexamethasone is preferred for Non-IgM type monoclonal protein kidney deposition, like in this case. Bortezomib and Thalidomide-based chemotherapy have been promising in recent research. For IgM type monoclonal protein deposition, Rituximab alone or in combination with cyclophosphamide and dexamethasone are used. This patient was not a good candidate for corticosteroid and chemotherapy or stem cell transplant due to old age (>77 years) and poor functional status, therefore, was started on hemodialysis. Following dialysis, improvement in renal function and general clinical condition was evident. The prognostic factors include age, degree of renal insufficiency at presentation affecting the renal prognosis, underlying hematologic disorder and extrarenal LC deposition. In this case, despite hemodialysis, long term survival and prognosis remain poor due to her inability to tolerate chemotherapy.
| Identifer | oai:union.ndltd.org:ETSU/oai:dc.etsu.edu:asrf-1106 |
| Date | 05 April 2018 |
| Creators | Singh, Kanwardeep, Sriramoju, Vindhya, Singal, Sakshi, Spradling, Elnora N, Zafar, Rabia |
| Publisher | Digital Commons @ East Tennessee State University |
| Source Sets | East Tennessee State University |
| Detected Language | English |
| Type | text |
| Format | application/pdf |
| Source | Appalachian Student Research Forum |
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