Abstract: SA-PO222
Myeloma Kidney with C3 Deposition
Session Information
- Trainee Case Reports - V
October 27, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Reports
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Khan, Asif, Staten Island University Hospital, Brooklyn, New York, United States
- El-Charabaty, Elie, Staten Island University Hospital, Brooklyn, New York, United States
- El Sayegh, Suzanne E., Staten Island University Hospital, Brooklyn, New York, United States
Introduction
Renal complications of multiple myeloma (MM) include monoclonal immunoglobulin deposition disease and myeloma cast nephropathy. Up to 50% of MM patients present with renal impairment at diagnosis, 20% may present with acute kidney injury (AKI) and 10% require dialysis. In C3 glomerulopathy, which is one of the complement-mediated types of Membranoproliferative Glomerulonephritis (MPGN), defects in complement regulatory proteins promotes the excessive activation of the alternative pathway.
Case Description
A 59-year-old man with a two-year history of hypertension presented with generalized weakness for the past 3 months. Physical examination reveals a well-appearing male with a bp 137/77 mmHg, pulse 103 bpm, with fairly normal physical findings. On admission: Hb 9.1 g/dl; RBC 1.45 mil/mm3; WBC 3.2 th/mm3; plt 94 th/mm3; BUN 94 mg/dl; Cr 10.47 mg/dL (baseline Cr 1.5 mg/dL); BUN/Cr 8.77; FeNa 8.7%; Na 130 mEq/L; K 6.2 mEq/L; HCO3 14 mEq/L; Urine analysis showed 2+ protein with bland urine sediment and microscopic hematuria. 24-hour urine protein was 2g/day. Renal ultrasound showed 0.6 cm right renal cyst.
The patient was admitted to ICU for hyperkalemia with AKI and was consequently hemodialyzed. Serologies were notable for low levels of C3 (46 mg/dL) with normal C4 were observed. Immunofixation by Electrophoresis of both serum and urine showed free Lambda band present. Serum plasma electrophoresis showed two M-spikes: Lambda light chains and IgG Lambda. Free kappa/lambda ratio <0.01. Urine electrophoresis showed three monoclonal bands. Serum levels of IgG 2500 mg/dl. A renal biopsy was performed and cast nephropathy was identified with mesangial staining for C3. Bone marrow biopsy showed CD 56 positive Plasma Cell Myeloma. MM was diagnosed with IgG lambda cast nephropathy and ESRD on dialysis with pancytopenia.
He was treated with Velcade, Cytoxan, and dexamethasone. SPEP status post chemotherapy showed two M-spikes with improvement of renal function and normal C3 levels. At 4 months post-biopsy, the patient remained dependent on hemodialysis therapy and required multiple blood transfusions.
Discussion
We hypothesize in monoclonal gammopathy induced C3 glomerulopathy, paraprotein itself is acting as a trigger that activates and dysregulates the AC pathway systemically. Thus, it is highly feasible to tailor the treatment to reduce the amount of paraproteins in C3 glomerulopathy associated with Myeloma kidney.