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 va...
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| Format: | Article |
| Language: | English |
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BMC
2022-08-01
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| Series: | BMC Nephrology |
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| Online Access: | https://doi.org/10.1186/s12882-022-02901-9 |
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| author | Gabriel Brayan Gutiérrez-Peredo José César Batista Oliveira Filho Iris Montaño-Castellón Andrea Jimena Gutiérrez-Peredo Edvan de Queiroz Crusoé Dimitri Gusmao-Flores |
| author_facet | Gabriel Brayan Gutiérrez-Peredo José César Batista Oliveira Filho Iris Montaño-Castellón Andrea Jimena Gutiérrez-Peredo Edvan de Queiroz Crusoé Dimitri Gusmao-Flores |
| author_sort | Gabriel Brayan Gutiérrez-Peredo |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-13T13:04:49Z |
| format | Article |
| id | doaj.art-05017c25524e47c3af9ffe2282672395 |
| institution | Directory Open Access Journal |
| issn | 1471-2369 |
| language | English |
| last_indexed | 2024-04-13T13:04:49Z |
| publishDate | 2022-08-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Nephrology |
| spelling | doaj.art-05017c25524e47c3af9ffe22826723952022-12-22T02:45:48ZengBMCBMC Nephrology1471-23692022-08-012311810.1186/s12882-022-02901-9Lambda light chain-induced monoclonal gammopathy of renal significance, manifesting with Fanconi Syndrome and osteomalaciaGabriel Brayan Gutiérrez-Peredo0José César Batista Oliveira Filho1Iris Montaño-Castellón2Andrea Jimena Gutiérrez-Peredo3Edvan de Queiroz Crusoé4Dimitri Gusmao-Flores5University Hospital Professor Edgard SantosUniversity Hospital Professor Edgard SantosUniversity Hospital Professor Edgard SantosUniversity Hospital Professor Edgard SantosUniversity Hospital Professor Edgard SantosUniversity Hospital Professor Edgard SantosAbstract 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.https://doi.org/10.1186/s12882-022-02901-9Monoclonal Gammopathy of Renal SignificanceFanconi SyndromeType 2 tubular acidosisNephrology |
| spellingShingle | Gabriel Brayan Gutiérrez-Peredo José César Batista Oliveira Filho Iris Montaño-Castellón Andrea Jimena Gutiérrez-Peredo Edvan de Queiroz Crusoé Dimitri Gusmao-Flores Lambda light chain-induced monoclonal gammopathy of renal significance, manifesting with Fanconi Syndrome and osteomalacia BMC Nephrology Monoclonal Gammopathy of Renal Significance Fanconi Syndrome Type 2 tubular acidosis Nephrology |
| title | Lambda light chain-induced monoclonal gammopathy of renal significance, manifesting with Fanconi Syndrome and osteomalacia |
| title_full | Lambda light chain-induced monoclonal gammopathy of renal significance, manifesting with Fanconi Syndrome and osteomalacia |
| title_fullStr | Lambda light chain-induced monoclonal gammopathy of renal significance, manifesting with Fanconi Syndrome and osteomalacia |
| title_full_unstemmed | Lambda light chain-induced monoclonal gammopathy of renal significance, manifesting with Fanconi Syndrome and osteomalacia |
| title_short | Lambda light chain-induced monoclonal gammopathy of renal significance, manifesting with Fanconi Syndrome and osteomalacia |
| title_sort | lambda light chain induced monoclonal gammopathy of renal significance manifesting with fanconi syndrome and osteomalacia |
| topic | Monoclonal Gammopathy of Renal Significance Fanconi Syndrome Type 2 tubular acidosis Nephrology |
| url | https://doi.org/10.1186/s12882-022-02901-9 |
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