Lambda light chain-induced monoclonal gammopathy of renal significance, manifesting with Fanconi Syndrome and osteomalacia

Abstract

Background Monoclonal gammopathy of renal significance (MGRS) encompasses a heterogeneous group of kidney diseases in which a monoclonal immunoglobulin secreted by a clone of B cells or plasma cells causes kidney damage without meeting the hematological criteria for malignancy. Among the various forms of involvement, MGRS can manifest as a proximal tubule disorder, such as Fanconi syndrome (FS), characterized by urinary loss of phosphate, glucose, amino acids, uric acid and bicarbonate. Few cases of MGRS have been described in the literature, manifesting as FS and monoclonal production of lambda light chains, almost all of which are secondary to the production of kappa light chains. Case presentation Here we report a clinical case of a 45-year-old Brazilian male, African descent, with proximal weakness of the lower limbs, whose initial assessment showed a urine summary with the presence of proteinuria and glycosuria without hyperglycemia, associated with mild worsening of renal function, hypouricemia, hypocalcemia and phosphaturia. Evolution was characterized by a MGRS manifesting as FS and osteomalacia. Conclusion The diagnosis of MGRS is not always easy, it requires knowledge of the clinical characteristics, diagnostic criteria and prognosis of each case. Therefore, all possible efforts should be made for multidisciplinary diagnosis.

Successful autologous stem cell transplantation for light chain proximal tubulopathy with severe kidney injury

“…Previous studies have not supported the use of chemotherapy drugs, such as alkylating agents, because of their ineffectiveness in improving renal function and adverse effects, such as secondary malignancy. 5 However, as new drugs for multiple myeloma have become available, 11 case reports and 4 case series have shown the efficacy of chemotherapy, particularly bortezomib‐based regimens and ASCT to improve renal and tubular function 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 (summarized in Tables 2 and 3 ). Although these studies comprised a small case series and the definition of the renal outcome was variable, all reports showed that chemotherapy improved renal function or delayed the progression to end‐stage kidney disease, compared with that in the non‐chemotherapy group (Table 2 ).…”

Successful autologous stem cell transplantation for light-chain proximal tubulopathy with severe kidney injury.

“…Previous studies did not support the use of chemotherapy such as alkylating agents because of its ineffectiveness in improving renal function and adverse effects such as secondary malignancy [5]. However, as new drugs for multiple myeloma have become available, 11 case reports and 4 case series have shown the efficacy of chemotherapy, particularly bortezomib-based regimens and ASCT to improve renal and tubular function [12][13][14][15][16][17][18][19][20][21][22] (summarized in Tables 2 and 3). Although these studies comprised a small case series, and the definition of the renal outcome was variable, all reports showed that chemotherapy improved renal function or delayed the progression to end-stage kidney disease, compared with that in the non-chemotherapy group (Table 2).…”

Comparison of Urinary Protein/Creatinine Ratio as an Alternative to 24-h Proteinuria in Lupus Nephritis: TUNARI Study

“…Clinical identification of LN can be challenging, being commonly discovered after careful examination of urine and renal function tests [1,3]. Monitoring the activity and/or control of LN is essential to prevent the progression of chronic kidney disease, which can have a negative impact on quality of life, functional dependence, comorbidities, and mortality risk [4][5][6].…”