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Monoclonal globulin disease of renal significance (MGRS) refers to B lymphocyte and plasma cell proliferative diseases that cause paraproteinemia renal damage, but it cannot reach multiple myeloma (MM), Waldenstrom's macroglobulinemia (WM) ), the diagnostic criteria of chronic lymphocytic leukemia (CLL) or malignant lymphoma.
If MGRS patients are not treated, they can lead to kidney failure and even require transplantation or dialysis.
At the First National Lymphocytic Disease Academic Conference of the Chinese Medical Association and the 2021 International Lymphoma Update Symposium, Professor Liu Qinhua from the First Affiliated Hospital of Anhui Medical University introduced the diagnosis and clinical characteristics of MGRS.
Organized as follows, for readers.
The definition of MGRS and the mechanism of kidney damage 01 The definition of MGRS The International Kidney and Monoclonal Immunoglobulin Working Group (IKMG) first proposed the concept and diagnostic criteria of MGRS in 2012, that is, M immunoglobulin (MIg) <30g/L, Bone marrow plasma cells (BMPC) <10%, kidney damage caused by Mlg (with or without other organ damage).
In 2019, IKMG redefined MGRS as the clonal proliferation of B cells or plasma cells that did not meet the diagnostic criteria for hematological malignancies, but the monoclonal immunoglobulins produced directly or indirectly caused kidney damage.
After the definition is updated, in addition to the common kidney damage associated with monoclonal immunoglobulin disease (MGUS) of unknown significance, kidney damage caused by smoking MM, smoking WM, and monoclonal B lymphocytosis (MBL) It also belongs to the category of MGRS.
In addition, low-invasive CLL and certain indolent lymphomas such as marginal zone lymphoma, mantle cell lymphoma, and MALT lymphoma are also classified as MGRS when they cause kidney damage.
Professor Liu Qinhua said that the above-mentioned diseases only require close monitoring when there is no kidney involvement, and active treatment is required once monoclonal immunoglobulin-related kidney damage occurs.
02 The mechanism of MGRS kidney damage Professor Liu Qinhua said that the mechanism of MGRS kidney damage is divided into direct mechanism and indirect damage mechanism.
The direct mechanism refers to the damage of MIg toxicity and deposition to the structure of kidney tissue, mainly including proliferative nephritis with monoclonal immunoglobulin deposition (PGNMID), immune barbed glomerulopathy, fibroid glomerulopathy and other glomerular damage ; Tubular nephropathy, proximal tubular disease and other renal tubular damage; amyloidosis, monoclonal immunoglobulin deposition disease (MIDD) and other glomerular, renal tubular, vascular damage and lens storage disease and other mechanisms.
The indirect damage mechanism refers to Mlg mimics autoantibodies, stimulates cytokines (such as mimic anti-H factor, anti-PLA2R) or MIg induces the release of vascular endothelial growth factor VEGF, including C3 glomerulopathy, membranous proliferative glomerulonephritis ( MPGN), membranous nephropathy and other glomerular damage and atypical hemolytic uremic syndrome (HUS) and other vascular damage.
Diagnosis and differential diagnosis of MGRS The diagnosis of MGRS depends on renal pathology.
For suspected cases of MGRS, such as the presence of M protein with unexplained renal damage, a small amount of proteinuria, and an increased proportion of lymphocytes, it is necessary to consult a nephrologist.
When the patient appears Non-diabetic nephrotic syndrome, non-ischemic cardiomyopathy with myocardial hypertrophy, hepatomegaly with elevated alkaline phosphatase, chronic inflammatory demyelinating polyneuropathy with Mlg or suspected light chain amyloidosis, Need to confirm the diagnosis by kidney puncture.
For patients unable to undergo renal puncture, lip line biopsy and abdominal fat biopsy are recommended.
Professor Liu Qinhua pointed out that after determining that the kidney damage is caused by monoclonal immunoglobulin, it is necessary to further determine the consistency of the circulating M protein and the protein deposited in the kidney.
The diagnosis of MGRS can only be established when the monoclonal immunoglobulin deposited in the kidney is the same as that in the blood and/or urine.
In addition.
Professor Liu Qinhua emphasized that MGRS needs to be differentiated from other MIg-related diseases.
If MM, WM, advanced CLL or malignant lymphoma can be diagnosed, it is no longer called MGRS.Clinical features and treatment strategies of MGRS 01 Clinical features of MGRS MGRS patients are mostly middle-aged and elderly, and most of them are older than 50 years old.
AL amyloidosis is the main renal pathological type of MGRS, followed by monoclonal immunoglobulin deposition nephropathy .
The first symptoms of MGRS are mainly edema, foamy urine, abnormal physical examination, chest tightness and skin rash.
The clinical manifestations of MGRS include facial and lower limb edema, proteinuria, occult blood in urine, increased creatinine, low protein, increased uric acid, anemia, electrolyte disorders, cardiac insufficiency, nephrotic syndrome, abnormal cardiac color Doppler ultrasound, etc.
The cell sources of MGRS-related clones are mainly plasma cell clones, followed by B cell clones, and a small part are plasma+B cell clones.
02MGRS treatment strategy Professor Liu Qinhua said that based on the clinical characteristics of MGRS, treatment needs to be formulated based on the cell source of MGRS-related clones.
If the source is a plasma cell clone, the main reference is the treatment plan for immunoglobulin light chain (AL) amyloidosis, such as bortezomib combined with dexamethasone; if the source is a B cell clone, the main treatment plan is rituximab Monoclonal antibody-based therapy; secondary kidney disease requires symptomatic treatment.
Professor Liu Qinhua also pointed out that before treatment, renal function assessment (proteinuria, kidney damage type, urine protein 24h quantification, etc.
) and cardiac function assessment (cardiac ultrasound, cardiac MRI, electrocardiogram, NT-proBNP, BNP, etc.
) are needed for MGRS patients before treatment.
Etc.
), M protein detection (serum free light chain, blood/urine IFE, serum protein electrophoresis, etc.
) and plasma cell/B cell proliferation detection.
In the evaluation of the efficacy of MGRS treatment, it can be based on the organ response and hematological response criteria of AL amyloidosis.
Summary Professor Liu Qinhua concluded that MGRS belongs to the intersection of kidney disease and hematological disease.
Kidney damage is directly or indirectly caused by monoclonal immunoglobulins produced by hematological diseases.
The diagnosis of MGRS relies on precise renal pathology.
M protein can deposit in different parts of the kidney and cause different damages.
The related renal damage is very heterogeneous. The choice of treatment plan for MGRS should be based on the patient's pathological type, clinical manifestations, risk of renal damage progression, extrarenal manifestations and severity, and the safety of drugs in patients with renal damage.
Professor Liu Qinhua, Associate Chief Physician, Doctor of Medicine, Department of Hematology, First Affiliated Hospital of Anhui Medical University, Member and Secretary, Hematology Branch of Anhui Medical Association, Deputy Leader of Thrombosis and Hemostasis Group, Hematology Branch of Anhui Medical Association, Hematology, Anhui Medical Association Member of the Lymphoma Myeloma Group, Director of the Hematology Branch of the Anhui General Practitioners Association, currently specializes in the diagnosis and treatment of lymphoma and myeloma.
He has been engaged in the specialty of hematology for nearly 20 years, and has rich clinical experience in the diagnosis and treatment of common, frequently-occurring, intractable, and critically ill hematological diseases.
Good at diagnosis and treatment of diseases such as lymphoma and myeloma.
Poke "read the original text" and we will make progress together
If MGRS patients are not treated, they can lead to kidney failure and even require transplantation or dialysis.
At the First National Lymphocytic Disease Academic Conference of the Chinese Medical Association and the 2021 International Lymphoma Update Symposium, Professor Liu Qinhua from the First Affiliated Hospital of Anhui Medical University introduced the diagnosis and clinical characteristics of MGRS.
Organized as follows, for readers.
The definition of MGRS and the mechanism of kidney damage 01 The definition of MGRS The International Kidney and Monoclonal Immunoglobulin Working Group (IKMG) first proposed the concept and diagnostic criteria of MGRS in 2012, that is, M immunoglobulin (MIg) <30g/L, Bone marrow plasma cells (BMPC) <10%, kidney damage caused by Mlg (with or without other organ damage).
In 2019, IKMG redefined MGRS as the clonal proliferation of B cells or plasma cells that did not meet the diagnostic criteria for hematological malignancies, but the monoclonal immunoglobulins produced directly or indirectly caused kidney damage.
After the definition is updated, in addition to the common kidney damage associated with monoclonal immunoglobulin disease (MGUS) of unknown significance, kidney damage caused by smoking MM, smoking WM, and monoclonal B lymphocytosis (MBL) It also belongs to the category of MGRS.
In addition, low-invasive CLL and certain indolent lymphomas such as marginal zone lymphoma, mantle cell lymphoma, and MALT lymphoma are also classified as MGRS when they cause kidney damage.
Professor Liu Qinhua said that the above-mentioned diseases only require close monitoring when there is no kidney involvement, and active treatment is required once monoclonal immunoglobulin-related kidney damage occurs.
02 The mechanism of MGRS kidney damage Professor Liu Qinhua said that the mechanism of MGRS kidney damage is divided into direct mechanism and indirect damage mechanism.
The direct mechanism refers to the damage of MIg toxicity and deposition to the structure of kidney tissue, mainly including proliferative nephritis with monoclonal immunoglobulin deposition (PGNMID), immune barbed glomerulopathy, fibroid glomerulopathy and other glomerular damage ; Tubular nephropathy, proximal tubular disease and other renal tubular damage; amyloidosis, monoclonal immunoglobulin deposition disease (MIDD) and other glomerular, renal tubular, vascular damage and lens storage disease and other mechanisms.
The indirect damage mechanism refers to Mlg mimics autoantibodies, stimulates cytokines (such as mimic anti-H factor, anti-PLA2R) or MIg induces the release of vascular endothelial growth factor VEGF, including C3 glomerulopathy, membranous proliferative glomerulonephritis ( MPGN), membranous nephropathy and other glomerular damage and atypical hemolytic uremic syndrome (HUS) and other vascular damage.
Diagnosis and differential diagnosis of MGRS The diagnosis of MGRS depends on renal pathology.
For suspected cases of MGRS, such as the presence of M protein with unexplained renal damage, a small amount of proteinuria, and an increased proportion of lymphocytes, it is necessary to consult a nephrologist.
When the patient appears Non-diabetic nephrotic syndrome, non-ischemic cardiomyopathy with myocardial hypertrophy, hepatomegaly with elevated alkaline phosphatase, chronic inflammatory demyelinating polyneuropathy with Mlg or suspected light chain amyloidosis, Need to confirm the diagnosis by kidney puncture.
For patients unable to undergo renal puncture, lip line biopsy and abdominal fat biopsy are recommended.
Professor Liu Qinhua pointed out that after determining that the kidney damage is caused by monoclonal immunoglobulin, it is necessary to further determine the consistency of the circulating M protein and the protein deposited in the kidney.
The diagnosis of MGRS can only be established when the monoclonal immunoglobulin deposited in the kidney is the same as that in the blood and/or urine.
In addition.
Professor Liu Qinhua emphasized that MGRS needs to be differentiated from other MIg-related diseases.
If MM, WM, advanced CLL or malignant lymphoma can be diagnosed, it is no longer called MGRS.Clinical features and treatment strategies of MGRS 01 Clinical features of MGRS MGRS patients are mostly middle-aged and elderly, and most of them are older than 50 years old.
AL amyloidosis is the main renal pathological type of MGRS, followed by monoclonal immunoglobulin deposition nephropathy .
The first symptoms of MGRS are mainly edema, foamy urine, abnormal physical examination, chest tightness and skin rash.
The clinical manifestations of MGRS include facial and lower limb edema, proteinuria, occult blood in urine, increased creatinine, low protein, increased uric acid, anemia, electrolyte disorders, cardiac insufficiency, nephrotic syndrome, abnormal cardiac color Doppler ultrasound, etc.
The cell sources of MGRS-related clones are mainly plasma cell clones, followed by B cell clones, and a small part are plasma+B cell clones.
02MGRS treatment strategy Professor Liu Qinhua said that based on the clinical characteristics of MGRS, treatment needs to be formulated based on the cell source of MGRS-related clones.
If the source is a plasma cell clone, the main reference is the treatment plan for immunoglobulin light chain (AL) amyloidosis, such as bortezomib combined with dexamethasone; if the source is a B cell clone, the main treatment plan is rituximab Monoclonal antibody-based therapy; secondary kidney disease requires symptomatic treatment.
Professor Liu Qinhua also pointed out that before treatment, renal function assessment (proteinuria, kidney damage type, urine protein 24h quantification, etc.
) and cardiac function assessment (cardiac ultrasound, cardiac MRI, electrocardiogram, NT-proBNP, BNP, etc.
) are needed for MGRS patients before treatment.
Etc.
), M protein detection (serum free light chain, blood/urine IFE, serum protein electrophoresis, etc.
) and plasma cell/B cell proliferation detection.
In the evaluation of the efficacy of MGRS treatment, it can be based on the organ response and hematological response criteria of AL amyloidosis.
Summary Professor Liu Qinhua concluded that MGRS belongs to the intersection of kidney disease and hematological disease.
Kidney damage is directly or indirectly caused by monoclonal immunoglobulins produced by hematological diseases.
The diagnosis of MGRS relies on precise renal pathology.
M protein can deposit in different parts of the kidney and cause different damages.
The related renal damage is very heterogeneous. The choice of treatment plan for MGRS should be based on the patient's pathological type, clinical manifestations, risk of renal damage progression, extrarenal manifestations and severity, and the safety of drugs in patients with renal damage.
Professor Liu Qinhua, Associate Chief Physician, Doctor of Medicine, Department of Hematology, First Affiliated Hospital of Anhui Medical University, Member and Secretary, Hematology Branch of Anhui Medical Association, Deputy Leader of Thrombosis and Hemostasis Group, Hematology Branch of Anhui Medical Association, Hematology, Anhui Medical Association Member of the Lymphoma Myeloma Group, Director of the Hematology Branch of the Anhui General Practitioners Association, currently specializes in the diagnosis and treatment of lymphoma and myeloma.
He has been engaged in the specialty of hematology for nearly 20 years, and has rich clinical experience in the diagnosis and treatment of common, frequently-occurring, intractable, and critically ill hematological diseases.
Good at diagnosis and treatment of diseases such as lymphoma and myeloma.
Poke "read the original text" and we will make progress together
