Lambda Light Chain Myeloma with Oliguric Cast Nephropathy and Remission with Bortezomib, Doxorubicin and Dexamethasone

A Sharma,B Poudyal,R Hada,R Khatri

doi:10.31729/jnma.378

Abstract

Cast nephropathy is one of the major causes of renal failure in patients with multiple myeloma resulting from precipitation of free light chains inside the tubules. Timely diagnosis and treatment confers a better prognosis though around 10% of patients with cast nephropathy remain dialysis dependent in spite of treatment. We report the clinical course and outcome of a patient presenting with acute kidney injury and oliguria, preceded by acute gastroenteritis and intake of Chinese medications and dialysis dependent state for eight weeks. Kidney biopsy revealed cast nephropathy with lambda light chain restriction and severe tubular injury. Serum protein electrophoresis was normal with no "M spike" but serum free light chain ratio was altered with very high lambda and normal kappa light chain levels. Bone marrow biopsy showed >85% atypical plasma cells. Haemodialysis was continued and chemotherapy with bortezomib, doxorubicin and dexamethasone was started. Kidney function gradually improved with discontinuation of dialysis after 1 month and complete remission of acute kidney injury and myeloma in 4 months of chemotherapy.

Highlights

OPEN ACCESSLambda Light Chain Myeloma with Oliguric Cast Nephropathy and Remission with Bortezomib, Doxorubicin and Dexamethasone
The major pathology found in myeloma patients with azotemia is cast nephropathy characterized by the presence of fractured, waxy tubular casts formed by precipitation of excessively filtered free light chains with Tamm-Horsfall protein resulting in tubular obstruction, direct tubular injury and severe Acute Kidney Injury (AKI).[6,7,8]
Though the mortality of cast nephropathy needing dialysis is significantly high,[5,6] we report a case of Correspondence: Dr Rajani Hada, Department of Nephrology, National Academy of Medical Sciences, Bir Hospital, Kathmandu, Nepal

Summary

OPEN ACCESS

Lambda Light Chain Myeloma with Oliguric Cast Nephropathy and Remission with Bortezomib, Doxorubicin and Dexamethasone. Hada R,1 Poudyal B,2 Sharma A,3 Khatri R4 1Department of Nephrology, National Academy of Medical Sciences, Bir Hospital and Blue Cross Hospital, Kathmandu, Nepal, 2Department of Clinical Haematology, Civil Service Hospital and Blue Cross Hospital, Kathmandu Nepal, 3Department of Histopathology, National Reference Laboratory, Dr Lal Path Labs Pvt Ltd, New Delhi, India, 4Department of Medicine, Shree Birendra Hospital and Blue Cross Hospital, Kathmandu Nepal

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