Abstract: SA-PO227
Plasmablastic Lymphoma Associated with IgD Lambda Light Chain Monoclonal Gammopathy and Severe AKI
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
- 102 AKI: Clinical, Outcomes, and Trials
Authors
- Abramson, Matthew, New York Presbyterian, New York, New York, United States
- Glezerman, Ilya, Memorial Sloan Kettering Cancer Center, New York, New York, United States
Introduction
Light chain cast nephropathy is the most common pattern of acute kidney injury in patients with multiple myeloma, seen in up to 47.5% of cases. Free light chains (FLC) are freely filtered and undergo endocytosis by proximal tubular cells via apical receptors. When these receptors become saturated, the resultant load of urinary free FLC co-aggregate with uromodulin causing precipitation and obstruction of the distal tubules, and inducing an inflammatory response. Most cases result from a single plasma clone of multiple myeloma. 2% of myeloma cases are of the IgD variant; only 5 cases have been reported to cause light chain cast nephropathy, and none as a result of lymphoma. This report describes the first case of presumed IgD lambda light chain cast nephropathy due to plasmablastic lymphoma.
Case Description
74 year-old man status-post nephrectomy at age 2, history of diffuse large B cell lymphoma status post R-CHOP in 2016 attaining complete remission, recent biopsy of new perisplenic lymph node revealing plasmablastic lymphoma, who presented with anorexia and decreased urine output. Nephrology was consulted for acute kidney injury (AKI) with creatinine of 9.7mg/dL (baseline of 1.2mg/dL).
He was found to have an IgD monoclonal gammopathy (1.4 g/dL), increasing serum lambda light chains (from 91mg/dL 3 weeks prior to 624mg/dL), and a kappa:lambda ratio of <0.01. 24-hour urine protein was 2.39g with M-spike of 1.65g and urinary immune-fixation positive for IgD lambda light chains. There was no obstruction on renal ultrasound. Bone marrow biopsy showed 0.2% lambda restricted clone, but no plasmacytosis. Although kidney biopsy was not obtained, the presumed diagnosis was IgD lambda light chain cast nephropathy. He was given intravenous hydration followed by cyclophosphamide, bortezomib, and dexamethasone. Serum creatinine improved rapidly to 3.1 mg/dL, serum lambda FLC improved to 40 mg/dL over the following week and patient was discharged home.
Discussion
Our patient developed severe AKI in the setting of IgD lambda FLC gammopathy likely leading to the development of cast nephropathy. Multiple myeloma literature reports up to 80% chance of renal recovery in patients with 60% reduction of serum FLC by 21 days. This patient had a similar remarkable response to standard myeloma therapy although his underlying pathology was plasmablastic lymphoma.