Abstract
Crystalline light chain cast nephropathy is a rare distinct morphologic variant of light chain cast nephropathy which is the most common renal lesion associated with multiple myeloma. It is often related to high myeloma tumor burden, severe acute kidney injury, and an unfavorable prognosis. A 79-year-old Japanese man was referred to our medical center with anemia, proteinuria, and acute exacerbation of the serum creatinine accompanying anuria. A renal biopsy showed crystalline cast filling the tubular lumens, injured tubular cells, and inflammatory cells infiltration of interstitium. Serum and urine immunofixation detected a monoclonal protein (IgA-λ and Bence-Jones Protein-λ, respectively), and bone marrow examination observed 64% of plasma cells. IgA-λ type multiple myeloma-associated crystalline light chain cast nephropathy and accompanying acute kidney injury were confirmed. Hydration and emergency hemodialysis were immediately introduced, and the treatment with bortezomib and dexamethasone was initiated. The patient showed successful recovery in renal manifestations. We suggest that early use with bortezomib-based therapy should be considered for patients with acute kidney injury caused by multiple myeloma-associated crystalline light chain cast nephropathy.