Spectrum of Monoclonal Gammopathy of Renal Significance (MGRS) in a Tertiary Care Hospital in India

Background: MGRS is a heterogeneous group of kidney disorders characterized by direct or indirect kidney injury caused by toxic monoclonal immunoglobulin that does not meet the current hematological malignancy criteria. The concept of MGRS was introduced in 2012 by IKMG. The diagnosis of MGRS is typically made by nephrologist through kidney biopsy. The followings are the Aims of the study: 1.Histopathological spectrum of MGRS patients, 2.Different clinical & pathological variable in MGRS patients, 3.Response to standard of care Methods: All MGRS patients (IKMG guidelines) attending IPGME& R hospital are included in this study over a period of 1st January'23 - December'23. All data are captured by using preformed proforma & analysed by SPSS software. Results: 15 patients were included. Median age - 52 years, (2 patients < 40 years). Male : Female 1:1. Diabetes was found in 5/15, one hypothyroid, 3 patients had hypertension, one patient had Urinary bladder Carcinoma. Presentations: Nephrotic syndrome - 8, RPRF - 3, AKI - 2, CKD3b -1 , CKD4-1. Peripheral blood - Eosinophilia ( Absolute count > 500/microlitre) found in 10. Renal biopsy: AL amyloidosis - 8, PGNMID - 3, MIDD ( LCDD) - 3 , C3 glomerulopathy - 1 Serum Protein Electrophoresis & Immunefixation: M band was detected in 60% of study population. AL amyloidosis (8) - M band in 5. Immunefixation showed IgG lambda in 6, 2 patients had IgG Kappa. PGNMID (3) - 1 had no M band & Immunefixation failed to detect any monoclonal intact immunoglobulin & or freelight chain. Renal biopsy showed IgG3kappa . Other two patients had no M band, Immunefixation showed IgG Kappa & lambda. LCDD (3) - one patient had no M band, but IgG Kappa was found in one patient, two patients showed IgG lambda. Serum Free light chain ratio was altered in 8, lambda dominance among 5, rest were Kappa . Conclusion: The MGRS group of kidney diseases often produces diagnostic challenges. So it requires multidisciplinary coordination. Cardiac death is common both pre and post therapy, particularly in AL amyloidosis patients. Therefore early & timely detection are required to improve overall & renal prognosis of patients & also reduces the chance of progression into overt hematological malignancy. Clone based therapy followed by HSCT may be curative. Funding: Government Support – Non-U.S.

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Journal of the American Society of Nephrology
Published
2024-10-01
DOI
https://doi.org/10.1681/asn.20243krme3y0
Primary Topic
Amyloidosis: Diagnosis, Treatment, Outcomes
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article
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article

Spectrum of Monoclonal Gammopathy of Renal Significance (MGRS) in a Tertiary Care Hospital in India

Atanu Pal, Tathagata Mukherjee, Rahul Mukherjee, Bibek Maulik
Journal of the American Society of Nephrology
Amyloidosis: Diagnosis, Treatment, Outcomes
article

Spectrum of Monoclonal Gammopathy of Renal Significance (MGRS) in a Tertiary Care Hospital in India

Atanu Pal, Tathagata Mukherjee, Rahul Mukherjee, Bibek Maulik
article en

Abstract

Background: MGRS is a heterogeneous group of kidney disorders characterized by direct or indirect kidney injury caused by toxic monoclonal immunoglobulin that does not meet the current hematological malignancy criteria. The concept of MGRS was introduced in 2012 by IKMG. The diagnosis of MGRS is typically made by nephrologist through kidney biopsy. The followings are the Aims of the study: 1.Histopathological spectrum of MGRS patients, 2.Different clinical & pathological variable in MGRS patients, 3.Response to standard of care Methods: All MGRS patients (IKMG guidelines) attending IPGME& R hospital are included in this study over a period of 1st January'23 - December'23. All data are captured by using preformed proforma & analysed by SPSS software. Results: 15 patients were included. Median age - 52 years, (2 patients < 40 years). Male : Female 1:1. Diabetes was found in 5/15, one hypothyroid, 3 patients had hypertension, one patient had Urinary bladder Carcinoma. Presentations: Nephrotic syndrome - 8, RPRF - 3, AKI - 2, CKD3b -1 , CKD4-1. Peripheral blood - Eosinophilia ( Absolute count > 500/microlitre) found in 10. Renal biopsy: AL amyloidosis - 8, PGNMID - 3, MIDD ( LCDD) - 3 , C3 glomerulopathy - 1 Serum Protein Electrophoresis & Immunefixation: M band was detected in 60% of study population. AL amyloidosis (8) - M band in 5. Immunefixation showed IgG lambda in 6, 2 patients had IgG Kappa. PGNMID (3) - 1 had no M band & Immunefixation failed to detect any monoclonal intact immunoglobulin & or freelight chain. Renal biopsy showed IgG3kappa . Other two patients had no M band, Immunefixation showed IgG Kappa & lambda. LCDD (3) - one patient had no M band, but IgG Kappa was found in one patient, two patients showed IgG lambda. Serum Free light chain ratio was altered in 8, lambda dominance among 5, rest were Kappa . Conclusion: The MGRS group of kidney diseases often produces diagnostic challenges. So it requires multidisciplinary coordination. Cardiac death is common both pre and post therapy, particularly in AL amyloidosis patients. Therefore early & timely detection are required to improve overall & renal prognosis of patients & also reduces the chance of progression into overt hematological malignancy. Clone based therapy followed by HSCT may be curative. Funding: Government Support – Non-U.S.

Journal of the American Society of NephrologyVol. 35(10S)
Institute of Post Graduate Medical Education and Research (IN)
Openalex Percentile: Top 83%
Amyloidosis: Diagnosis, Treatment, Outcomes
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