Nephrotic Syndrome from Primary Amyloidosis Can Be Linked with Multiple Myeloma: A Rare Presentation of a Case
- Glomerular Diseases: Podocyte Biology - II
November 04, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1403 Podocyte Biology
- Islam, Masuma, St Mary's Hospital, Waterbury, Connecticut, United States
- Hoopes, Raquel, Quinnipiac University, Hamden, Connecticut, United States
- Chowdhury, Monzurul Hasan, St Mary's Hospital, Waterbury, Connecticut, United States
Nephrotic syndrome from primary amyloidosis can co-exist with multiple myeloma on rare occasions. Early diagnosis and Appropriate management is crucial for the prognosis of these patients as the combination can reduce life expectancy.
A 61-year-old female with PMH of Type 2 DM, HTN was seen for nephrotic range proteinuria detected by PCP screening. Her DM and HTN have been well-controlled for over 10 years. She had no complaints. Physical examination was unremarkable. She has had microalbuminuria since 2017 but developed significant proteinuria and hypoalbuminuria over the last year. A renal ultrasound was done which showed normal kidneys. Laboratory tests showed normal kidney function.
Serum electrophoresis revealed monoclonal gammopathy and urine immunofixation showed monoclonal lambda plus IgG lambda fragments. Renal biopsy showed amyloidosis with randomly elevated fibrillary structure. Bone marrow biopsy showed mildly hypercellular marrow with 30% plasmacytosis. Flow cytometry showed lambda light chain.
The patient was diagnosed with asymptomatic multiple myeloma and amyloidosis presenting as nephrotic syndrome. She was treated with daratumumab plus CyBorD, which includes cyclophosphamide, bortezomib, and dexamethasone. Over several months, her serum albumin levels have increased and the lambda light chain has decreased.
Multiple myeloma (MM) is a malignant plasma cell disorder characterized by uncontrolled proliferation and infiltration of monoclonal plasma cells to the bone marrow. MM is observed concomitantly with amyloidosis in 12-15% of cases 1 . Amyloidosis refers to the abnormal deposition of immunoglobulin light chains throughout body tissues.
This case highlights a rare but possible scenario of asymptomatic multiple myeloma and amyloidosis that presented as nephrotic syndrome.
Diagnosing asymptomatic multiple myeloma and amyloidosis can be challenging, as patients may have no significant symptoms. Further research is needed to investigate which types of patients are at risk.
In this case, the patient was diagnosed following routine urine screening for diabetic nephropathy, emphasizing the importance of screening for these conditions. Treatment for asymptomatic multiple myeloma and amyloidosis largely overlaps, focusing on reducing abnormal proteins and managing complications such as renal damage.