Abstract: FR-PO257
Non-Proteinuric CKD: An Atypical Presentation of Multiple Myeloma
Session Information
- Onconephrology: From AKI to CKD and Everything in Between
November 03, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Onconephrology
- 1700 Onconephrology
Authors
- Pawly, Chrystel, Yale University Department of Internal Medicine, New Haven, Connecticut, United States
- Donati, Andrew, CT Kidney and HTN Specialists LLC, Waterbury, Connecticut, United States
Introduction
Light chain deposition disease (LCDD) is characterized by overproduction and deposition of monoclonal light chain immunoglobulins. LCDD usually has prominent kidney involvement; including proteinuria and kidney failure characterized by nodular glomerulosclerosis and tubular basement membrane thickening. Here we highlight an atypical presentation of biopsy proven kappa type LCDD without significant glomerular proteinuria in a patient found to have Kappa restricted Multiple myeloma (MM).
Case Description
Our patient is a 64-year-old male with medical history of asthma, hyperlipidemia, and tobacco use disorder (remission for 9 yr). He presented to nephrology clinic for evaluation of confirmed isolated elevation in creatinine of 2.1 (BUN 21) with normal CBC, electrolytes, total protein and albumin. History was negative for NSAID use, OTC supplements, frequent UTI or nephrolithiasis. There was no family history of CKD or ESKD. His only medication was Fluticasone-Vilanterol. His vital signs and physical exam were unremarkable. Urinalysis was negative for protein, but trace blood was noted. ANA, ANCA, C3/C4, RF, and viral hepatitis serologies were negative. UACR 10mcg/mg and UPCR
0.2mg/mg. SPEP was normal. Kappa FLC 273 mg/L, Lambda FLC 26.1 mg/L, Ratio 10.46. Renal imaging was notable for multiple bilateral cysts without evidence of enhancing lesion, stones or hydronephrosis. Renal biopsy was ultimately performed, and it revealed LCDD Kappa type, interstitial fibrosis/tubular atrophy of 70-80% with moderate arterio- and arteriolosclerosis. Bone marrow biopsy revealed normal cellular marrow with trilineage hematopoiesis and increased plasma cell (20% by CD 138 immunostaining). Flow cytometry revealed monoclonal kappa restricted plasma cell population. PET/CT was normal, implying that the kidneys were the sole site of the disease. He was started on cyclophosphamide, bortezomib and dexamethasone (CyBorD) and creatinine eventually improved. UACR increased slightly to 25mcg/mg.
Discussion
Kidney biopsy is almost always essential when the origin of CKD is unknown. Among patients newly diagnosed with MM, 20-50% have either AKI or CKD at the time of diagnosis. Early recognition and treatment of an underlying plasma cell disorder is important in preserving kidney function. This case highlights the importance of thorough workup for monoclonal gammopathy despite absence of typical presenting features.