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The 9th American Society of Hematology Oncology (SOHO) Annual Meeting will be held in Houston, USA on September 8-11, 2021
.
The SOHO Annual Conference is an international conference on hematological malignancies specially designed for clinicians, research scientists and related health professionals.
This year’s SOHO conference will continue to deliver high-quality content and focus on providing practitioners and practitioners in the field of hematological malignancies.
Scientists provide the latest information
.
A report published at this conference introduced how to identify and treat monoclonal gammopathy (MGRS) with renal significance
.
The editor now organizes the main content of the research as follows for the reference of readers
.
Monoclonal gammopathy with renal significance (MGRS) is a group of B-cell or plasma cell proliferative diseases that cause kidney damage through the production of monoclonal immunoglobulins
.
As the current diagnostic criteria for plasma cell disease, Waldenstrom's macroglobulinemia, and chronic lymphocytic leukemia (CLL) lack the relevant criteria for the kidney, it is necessary to define the blood system disease separately
.
According to the definition, MGRS is a hematological malignant disease that does not meet the definition of a malignant tumor or does not meet the treatment criteria, but due to end-organ damage, it cannot be diagnosed as an unexplained monoclonal gammopathy (MGUS)
.
Although most MGRS are not life-threatening except for immunoglobulin-associated (AIg) amyloidosis and monoclonal immunoglobulin deposition disease (MIDD) with extra-renal manifestations, kidney diseases secondary to MGRS may progress, leading to The death of some MGRS patients
.
Since MGUS is generally not recommended for treatment, this type of disease is not suitable to be classified as MGUS
.
Since these diseases require clonal-directed therapy to protect kidney function, these diseases are not suitable for MGUS, IgM-type MGUS, and low-risk CLL
.
Defining this type of disease as MGRS can be clearly distinguished from MGUS.
At the same time, clonal-oriented therapy can be used in treatment, and it does not conflict with existing diagnostic standards and guidelines
.
Most diagnosis of MGRS requires a kidney biopsy
.
In addition to light chain type (AL) amyloidosis that needs to be diagnosed by other tissues, and light chain proximal tubule disease with Fanconi syndrome that needs to be diagnosed by electrolyte abnormalities, most of the diagnosis of MGRS requires renal biopsy
.
40%-45% of monoclonal gammopathy patients undergoing renal biopsy have MGRS-related nephropathy
.
Immunofluorescence detection is one of the most important tests in renal biopsy.
MGRS-related diseases have three different test results: the most common is the monoclonal represented by light chain restriction (or only heavy chain restriction in heavy chain diseases) Immune globulin deposition; dominant complement (with very few immunoglobulins) deposition is more common in C3 glomerulopathy; thrombotic microangiopathy has no immune deposition
.
A series of kidney diseases are currently known to be associated with MGRS, including AIg amyloidosis, immune barbed-like glomerulopathy with monoclonal gammopathy, MIDD, proliferative glomerulonephritis with monoclonal immunoglobulin deposition (PGNMID) , Light chain proximal tubule disease (Fanconi syndrome), crystal storage histiocytosis, crystal globulinemia syndrome, C3 glomerulopathy with monoclonal gammopathy, and monoclonal gammopathy Disease-related thrombotic microangiopathy
.
In C3 glomerulopathy cases with monoclonal gammopathy, about half of monoclonal immunoglobulins cause complement disorders through nephritis factor or factor H, factor I, and CR1 antibodies
.
Thrombotic microangiopathy is a common pathological change in patients with POEMS syndrome
.
Recent studies have shown that thrombotic microangiopathy is the initial manifestation of patients with multiple myeloma or Waldenstrom's macroglobulinemia
.
Most MGRS-related kidney diseases are caused by plasma cell clones, but the proportion of plasma cell clones varies depending on the renal disease.
For example, plasma cell clones account for about 95% of AL amyloidosis and MIDD clones, but clones derived from CLL account for about immune 50% of tentacled glomerulonephritis clones
.
In addition, CD20+ cell clones were also observed in 50% of PGNMID patients, and about half of them also co-express CD38
.
In type I cryoglobulinemia, lymphoplasmacytic lymphoma clones are the most common
.
The main goal of MGRS treatment is to protect kidney function or prevent recurrence after kidney transplantation if the kidney cannot be saved
.
For patients with extrarenal manifestations such as systemic AIg amyloidosis or MIDD, attention should be paid to the safety of the patient’s life during treatment
.
Patients with irreversible advanced chronic kidney disease should postpone treatment until the patient meets the treatment criteria for their hematological diseases
.
Relevant studies have shown that in order to protect kidney function, patients need at least a very good partial remission
.
Therefore, compared with standard immunosuppressive therapy, clone-directed therapy is more suitable for MGRS patients
.
Bortezomib can effectively treat plasma cell cloned MGRS, and the anti-CD20 monoclonal antibody rituximab can be used for the treatment of CD20 cloned MGRS
.
Autologous hematopoietic stem cell transplantation (ASCT) consolidation therapy can further deepen disease remission and bring better curative effects for MIDD patients
.
Since most of these diseases are derived from hypoproliferative diseases, a limited course of treatment can achieve better curative effects.
This type of patients usually does not need maintenance therapy, and maintenance therapy is only used for relapsed patients
.
Except for AL amyloidosis, there are currently no relevant clinical trials for MGRS
.
A small phase II study explored the efficacy of daratumumab in the treatment of PGNMID
.
The results of the study showed that 90% of patients achieved renal remission after treatment with daratumomab, and about 30% of patients achieved complete renal remission
.
It is still necessary to carry out further clinical studies to confirm the efficacy of daratumumab in this type of patients
.
Reference source: 1.
Nelson Leung, et al.
2021 SOHO.
EXABS-122-MM.
Stamp "read the original text", we make progress together
.
The SOHO Annual Conference is an international conference on hematological malignancies specially designed for clinicians, research scientists and related health professionals.
This year’s SOHO conference will continue to deliver high-quality content and focus on providing practitioners and practitioners in the field of hematological malignancies.
Scientists provide the latest information
.
A report published at this conference introduced how to identify and treat monoclonal gammopathy (MGRS) with renal significance
.
The editor now organizes the main content of the research as follows for the reference of readers
.
Monoclonal gammopathy with renal significance (MGRS) is a group of B-cell or plasma cell proliferative diseases that cause kidney damage through the production of monoclonal immunoglobulins
.
As the current diagnostic criteria for plasma cell disease, Waldenstrom's macroglobulinemia, and chronic lymphocytic leukemia (CLL) lack the relevant criteria for the kidney, it is necessary to define the blood system disease separately
.
According to the definition, MGRS is a hematological malignant disease that does not meet the definition of a malignant tumor or does not meet the treatment criteria, but due to end-organ damage, it cannot be diagnosed as an unexplained monoclonal gammopathy (MGUS)
.
Although most MGRS are not life-threatening except for immunoglobulin-associated (AIg) amyloidosis and monoclonal immunoglobulin deposition disease (MIDD) with extra-renal manifestations, kidney diseases secondary to MGRS may progress, leading to The death of some MGRS patients
.
Since MGUS is generally not recommended for treatment, this type of disease is not suitable to be classified as MGUS
.
Since these diseases require clonal-directed therapy to protect kidney function, these diseases are not suitable for MGUS, IgM-type MGUS, and low-risk CLL
.
Defining this type of disease as MGRS can be clearly distinguished from MGUS.
At the same time, clonal-oriented therapy can be used in treatment, and it does not conflict with existing diagnostic standards and guidelines
.
Most diagnosis of MGRS requires a kidney biopsy
.
In addition to light chain type (AL) amyloidosis that needs to be diagnosed by other tissues, and light chain proximal tubule disease with Fanconi syndrome that needs to be diagnosed by electrolyte abnormalities, most of the diagnosis of MGRS requires renal biopsy
.
40%-45% of monoclonal gammopathy patients undergoing renal biopsy have MGRS-related nephropathy
.
Immunofluorescence detection is one of the most important tests in renal biopsy.
MGRS-related diseases have three different test results: the most common is the monoclonal represented by light chain restriction (or only heavy chain restriction in heavy chain diseases) Immune globulin deposition; dominant complement (with very few immunoglobulins) deposition is more common in C3 glomerulopathy; thrombotic microangiopathy has no immune deposition
.
A series of kidney diseases are currently known to be associated with MGRS, including AIg amyloidosis, immune barbed-like glomerulopathy with monoclonal gammopathy, MIDD, proliferative glomerulonephritis with monoclonal immunoglobulin deposition (PGNMID) , Light chain proximal tubule disease (Fanconi syndrome), crystal storage histiocytosis, crystal globulinemia syndrome, C3 glomerulopathy with monoclonal gammopathy, and monoclonal gammopathy Disease-related thrombotic microangiopathy
.
In C3 glomerulopathy cases with monoclonal gammopathy, about half of monoclonal immunoglobulins cause complement disorders through nephritis factor or factor H, factor I, and CR1 antibodies
.
Thrombotic microangiopathy is a common pathological change in patients with POEMS syndrome
.
Recent studies have shown that thrombotic microangiopathy is the initial manifestation of patients with multiple myeloma or Waldenstrom's macroglobulinemia
.
Most MGRS-related kidney diseases are caused by plasma cell clones, but the proportion of plasma cell clones varies depending on the renal disease.
For example, plasma cell clones account for about 95% of AL amyloidosis and MIDD clones, but clones derived from CLL account for about immune 50% of tentacled glomerulonephritis clones
.
In addition, CD20+ cell clones were also observed in 50% of PGNMID patients, and about half of them also co-express CD38
.
In type I cryoglobulinemia, lymphoplasmacytic lymphoma clones are the most common
.
The main goal of MGRS treatment is to protect kidney function or prevent recurrence after kidney transplantation if the kidney cannot be saved
.
For patients with extrarenal manifestations such as systemic AIg amyloidosis or MIDD, attention should be paid to the safety of the patient’s life during treatment
.
Patients with irreversible advanced chronic kidney disease should postpone treatment until the patient meets the treatment criteria for their hematological diseases
.
Relevant studies have shown that in order to protect kidney function, patients need at least a very good partial remission
.
Therefore, compared with standard immunosuppressive therapy, clone-directed therapy is more suitable for MGRS patients
.
Bortezomib can effectively treat plasma cell cloned MGRS, and the anti-CD20 monoclonal antibody rituximab can be used for the treatment of CD20 cloned MGRS
.
Autologous hematopoietic stem cell transplantation (ASCT) consolidation therapy can further deepen disease remission and bring better curative effects for MIDD patients
.
Since most of these diseases are derived from hypoproliferative diseases, a limited course of treatment can achieve better curative effects.
This type of patients usually does not need maintenance therapy, and maintenance therapy is only used for relapsed patients
.
Except for AL amyloidosis, there are currently no relevant clinical trials for MGRS
.
A small phase II study explored the efficacy of daratumumab in the treatment of PGNMID
.
The results of the study showed that 90% of patients achieved renal remission after treatment with daratumomab, and about 30% of patients achieved complete renal remission
.
It is still necessary to carry out further clinical studies to confirm the efficacy of daratumumab in this type of patients
.
Reference source: 1.
Nelson Leung, et al.
2021 SOHO.
EXABS-122-MM.
Stamp "read the original text", we make progress together
