Light-Chain Deposition Disease (LCDD) is an idiopathic condition associated with the extracellular deposition of monoclonal immunoglobulin light chains (predominantly the kappa type) in organ tissues with the kidney being most affected. It is one of the monoclonal gammopathies which are clinical conditions resulting from the increased production of monoclonal immunoglobulins or their fragments by abnormal plasma cells. It is often linked to a pre-existing plasma cell dyscrasia, including multiple myeloma or monoclonal gammopathy of unknown origin (MGUS). The plasma cells that are affected by the cancer form abnormal light chains that circulate in the blood.
Epidemiology
Currently, there is no accurate statistical data on the incidence rates of light-chain deposition disease (LCDD). Though, it is detected in approximately 5% of the patients who have passed away due to multiple myeloma. Major mortality and morbidity factors in LCDD are often associated with kidney-related ailment comprisinghypertension, nephrotic syndrome and progression to ESRD. Also, liver involvement can cause the liver to malfunction and ultimately produce hepatic failure.
Anatomy
Pathophysiology
Renal LCDD has distinct features at the microscopic level and these include nodular sclerosing glomerulonephritis on LM, diffuse linear light chain deposition on IFM, and electron-dense deposits on EM. It concerns the abnormal accumulation of kappa light chains in the kidneys, within glomerular tufts and tubular basement membranes, which may cause kidney impairment.
Specimens stained for kappa and lambda chains is essential for diagnosis and positivity of kappa chains are often observed along tubular basement membrane. Electron microscopy can be useful in the identification of LCDD from other types of amyloid light-chain (AL) amyloidosis which is mostly associated with lambda light chain.
The involvement of extra renal organs in LCDD is limited but organs which may be involved include the liver, heart, lungs and the peripheral nerves. The liver is another site which is affected in more than 90 percent of patients with diabetic nephropathy and may result in liver cirrhosis and liver failure. Some of the effects of systemic sclerosis on the heart are cardiomyopathy and congestive heart failure and, on the lung, lung disease. Peripheral neuropathy and in some cases even brain involvement has also been observed.
LCDD is most linked to multiple myeloma in patients, where it occurs in approximately 58% of patients. It may be diagnosed at the time of a suspected plasma cell neoplasm or as a manifestation of multiple myeloma at relapse. LCDD may also affect the other subtypes of B-cell lymphomas.
Etiology
Light-Chain Deposition Disease (LCDD) is primarily related to plasma cell dyscrasias, more commonly multiple myeloma and Monoclonal Gammopathy of Undetermined Significance (MGUS), in which aberrant plasma cells synthesize and release excessive monoclonal light chains that precipitate in tissues. It can also be associated with other B-cell lymphomas, namely, lymphoplasmacytic lymphoma and chronic lymphocytic leukemia. These conditions may be caused in part by genetic susceptibility, but environmental factors, chronic inflammation, and infections also may be involved, although these factors are not as well defined as genetic factors.
Genetics
Prognostic Factors
For instance, the median survival for patients with light-chain deposition disease (LCDD) is under 4 years. In the largest study to date, patients were followed for a median of 27 months; 57% developed uremia, and 59% had died. Of the predictors, patient age, the diagnosis of multiple myeloma, and extrarenal light-chain deposition. Most important, ESRD and need for dialysis did not adversely affect the prognosis.”
These factors include age, multiple myeloma or cast nephropathy, and the presence of extrarenal light chain deposition.
Given the fact that LCDD is rare, it remains difficult to identify the natural course of the disease or the effects of its treatment.
Clinical History
Age Group
The most common age group for LCDD is adults, and more specifically, those of middle to older age, in their 50s – 70s. It is not common in young persons especially at the early ages.
Physical Examination
End-organ dysfunction is frequent in LCDD and may dominate clinical manifestations of the disease in the form of hypertension, peripheral oedema, neuropathy or congestive heart failure. It is understood that approximately 50% of LCDD patients have nephrotic syndrome. However, the proteinuria is less than 1g/day in about 25% of cases because of Tubulointerstitial Involvement. Abnormal physical signs that may be looked for include new heart sounds and splenomegaly.
Age group
Associated comorbidity
Multiple myeloma
B-Cell lymphoma
Monoclonal Gammopathy of Undetermined Significance (MGUS)
Congestive heart failure
Associated activity
Acuity of presentation
Gradual Onset: LCDD typically has a gradual clinical course with the development of symptoms during months and years.
Chronic Presentation: Many patients are usually asymptomatic or have chronic complaints like swelling, high BP or slowly progressive renal failure.
Acute Exacerbations: There can be severe exacerbations in some cases, often connected with cardiac or renal failure; such states are always worsening the patient’s condition and may be life-threatening.
Differential Diagnoses
Familial Renal Amyloidosis
IgA Nephropathy
Light Chain-Associated Renal Disorders
Nephrotic Syndrome
Diabetic Nephropathy
Heart Failure
Immunoglobulin-Related Amyloidosis
Multiple Myeloma
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
Treatment of Underlying Plasma Cell Disorder:
Multiple Myeloma or Monoclonal Gammopathy of Undetermined Significance (MGUS):
Chemotherapy: Medications like bortezomib, lenalidomide, and dexamethasone are utilized in the treatment process with the aim of extirpating the aberrant plasma cells that produce the light chains.
Immunomodulatory Agents: Plasma cell dyscrasia could also be treated with the help of agents such as thalidomide and pomalidomide.
Targeted Therapy: ERAD components include proteasome inhibitors (e. g., carfilzomib) and monoclonal antibodies (e. g., daratumumab) that slow down the generation of light chains.
Stem Cell Transplant: Intensification with high-dose chemotherapy and autologous stem cell rescue could be used for intensification for selected patients with a view of inducing further disease remission.
Management of Renal Complications:
Control of Proteinuria: Renal management that includes the treatment with angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) with the purpose of reduction of proteinuria and protection of renal function.
Management of Hypertension B: antihypertensive agents that will help to lower the blood pressure to avoid further deterioration of renal function.
Renal Replacement Therapy: In case of AKI progression to advanced renal impairment or ESRD; dialysis or kidney transplantation may be necessary.
Symptomatic Management:
Edema Management: It should be also mentioned that diuretics are effective in case of fluid overload problem and peripheral edema.
Cardiac Symptoms: Use of cardio-selective drugs such as those that are used in the treatment of congestive heart failure or arrhythmias.
Supportive Care:
Nutritional Support: Low protein and heart healthy diets and nutrition management of renal function and cardiovascular disease.
Pain Management: Pain management following LCDD and its complications; the use of analgesics and other supportive measures.
Protein Intake: A low protein diet is also useful in decreasing kidney workload and in controlling proteinuria.
Sodium and Fluid Restriction: Restriction in salt intake as well as restriction in the intake of fluids reduces hypertension and edema respectively.
Heart-Healthy Diet: A diet restricted in saturated fat, cholesterol, and sodium enhances the cardiovascular health.
Lifestyle Changes:
Weight Management: Control of diet and exercise also plays a crucial role in weight control which is in support of the cardiovascular health and to minimise stress on the kidney.
Exercise: Likelihood of decreased exercise related to cardiovascular health and Chronic illnesses conditions are reduced through, moderate, regular exercise.
Smoking Cessation: Smoking cessation is beneficial in all aspects of health and especially in the cardiovascular health.
Role of Proteasome Inhibitors
Bortezomib (Velcade): It is employed to target the proteasome needed in the breakdown of proteins that control cell division and death. This has the effect of lowering the count of malignant plasma cells and diminishes the synthesis of monoclonal light chains.
Carfilzomib (Kyprolis): Like bortezomib, carfilzomib is another second-generation proteasome inhibitor which also aims at the target to decrease the plasma cells.
Role of Immunomodulatory Agents
Lenalidomide (Revlimid): Lenalidomide is an anti-angiogenic, anti-inflammatory, and immunomodulating agent. It diminishes the amount of abnormal plasma cells and aids in elevating the immune system functions in relation to killing cancerous cells.
Thalidomide: Thalidomide has relatively fewer applications compared to other drugs because of its side effects, but it has immunomodulatory effects that can assist in managing the proliferation of the malignant plasma cells.
Role of Corticosteroids
Dexamethasone: Dexamethasone being a corticosteroid is sometimes used in conjunction with other agents used for chemotherapy, to minimize inflammation and slowing the proliferation of malignant plasma cells. They are included in the standard therapy protocols for patients with plasma cell diseases.
Role of Monoclonal Antibodies
Daratumumab:It is a monoclonal antibody that binds to the CD38 receptor which is present on the surface of plasma cells. It is also useful in decreasing the number of malignant plasma cells and, as a result, the number of light chains produced is reduced.
Plasma Exchange: It is performed to eliminate light chains from the bloodstream with the aim of alleviating pathologic manifestations connected to the renal and extrarenal deposits. The plasma with light chains is separated from the patient’s blood and a machine returns the rest of blood components and pumps in plasma or albumin.
Kidney Biopsy: It is used in the diagnosis of LCDD to look for the presence of typical light chains deposits into the kidney tissue. Mostly, a small amount of tissue from the kidney is collected; this process is usually done using a needle inserted into the kidney with the help of images. Light microscopical, immunofluorescent and electron microscopical examinations of the tissue are then performed.
Approaches used in light chain deposition disease are broadly categorized into phases. First, the diagnosis and the initial evaluation are necessary and accomplished through biopsy and imaging to assess the involvement of the organ. The treatment phase is aimed to deal with the plasma cell dyrease with the help of chemotherapeutic drugs (bortezomib, lenalidomide), to treat the symptoms such as proteinuria and hypertension and to support with dietary/nutritional and lifestyle interventions. Response monitoring includes constant assessment of the efficacy of the treatment and modifying the therapy if necessary. Finally, patients with end-stage renal disease (ESRD) or cardiovascular problems are treated in the advanced phase of the disease and patient counseling and follow-up are performed. If required, the end-of-life or palliative care is offered, to improve the quality of life.
Light-Chain Deposition Disease (LCDD) is an idiopathic condition associated with the extracellular deposition of monoclonal immunoglobulin light chains (predominantly the kappa type) in organ tissues with the kidney being most affected. It is one of the monoclonal gammopathies which are clinical conditions resulting from the increased production of monoclonal immunoglobulins or their fragments by abnormal plasma cells. It is often linked to a pre-existing plasma cell dyscrasia, including multiple myeloma or monoclonal gammopathy of unknown origin (MGUS). The plasma cells that are affected by the cancer form abnormal light chains that circulate in the blood.
Currently, there is no accurate statistical data on the incidence rates of light-chain deposition disease (LCDD). Though, it is detected in approximately 5% of the patients who have passed away due to multiple myeloma. Major mortality and morbidity factors in LCDD are often associated with kidney-related ailment comprisinghypertension, nephrotic syndrome and progression to ESRD. Also, liver involvement can cause the liver to malfunction and ultimately produce hepatic failure.
Renal LCDD has distinct features at the microscopic level and these include nodular sclerosing glomerulonephritis on LM, diffuse linear light chain deposition on IFM, and electron-dense deposits on EM. It concerns the abnormal accumulation of kappa light chains in the kidneys, within glomerular tufts and tubular basement membranes, which may cause kidney impairment.
Specimens stained for kappa and lambda chains is essential for diagnosis and positivity of kappa chains are often observed along tubular basement membrane. Electron microscopy can be useful in the identification of LCDD from other types of amyloid light-chain (AL) amyloidosis which is mostly associated with lambda light chain.
The involvement of extra renal organs in LCDD is limited but organs which may be involved include the liver, heart, lungs and the peripheral nerves. The liver is another site which is affected in more than 90 percent of patients with diabetic nephropathy and may result in liver cirrhosis and liver failure. Some of the effects of systemic sclerosis on the heart are cardiomyopathy and congestive heart failure and, on the lung, lung disease. Peripheral neuropathy and in some cases even brain involvement has also been observed.
LCDD is most linked to multiple myeloma in patients, where it occurs in approximately 58% of patients. It may be diagnosed at the time of a suspected plasma cell neoplasm or as a manifestation of multiple myeloma at relapse. LCDD may also affect the other subtypes of B-cell lymphomas.
Light-Chain Deposition Disease (LCDD) is primarily related to plasma cell dyscrasias, more commonly multiple myeloma and Monoclonal Gammopathy of Undetermined Significance (MGUS), in which aberrant plasma cells synthesize and release excessive monoclonal light chains that precipitate in tissues. It can also be associated with other B-cell lymphomas, namely, lymphoplasmacytic lymphoma and chronic lymphocytic leukemia. These conditions may be caused in part by genetic susceptibility, but environmental factors, chronic inflammation, and infections also may be involved, although these factors are not as well defined as genetic factors.
For instance, the median survival for patients with light-chain deposition disease (LCDD) is under 4 years. In the largest study to date, patients were followed for a median of 27 months; 57% developed uremia, and 59% had died. Of the predictors, patient age, the diagnosis of multiple myeloma, and extrarenal light-chain deposition. Most important, ESRD and need for dialysis did not adversely affect the prognosis.”
These factors include age, multiple myeloma or cast nephropathy, and the presence of extrarenal light chain deposition.
Given the fact that LCDD is rare, it remains difficult to identify the natural course of the disease or the effects of its treatment.
Age Group
The most common age group for LCDD is adults, and more specifically, those of middle to older age, in their 50s – 70s. It is not common in young persons especially at the early ages.
End-organ dysfunction is frequent in LCDD and may dominate clinical manifestations of the disease in the form of hypertension, peripheral oedema, neuropathy or congestive heart failure. It is understood that approximately 50% of LCDD patients have nephrotic syndrome. However, the proteinuria is less than 1g/day in about 25% of cases because of Tubulointerstitial Involvement. Abnormal physical signs that may be looked for include new heart sounds and splenomegaly.
Multiple myeloma
B-Cell lymphoma
Monoclonal Gammopathy of Undetermined Significance (MGUS)
Congestive heart failure
Gradual Onset: LCDD typically has a gradual clinical course with the development of symptoms during months and years.
Chronic Presentation: Many patients are usually asymptomatic or have chronic complaints like swelling, high BP or slowly progressive renal failure.
Acute Exacerbations: There can be severe exacerbations in some cases, often connected with cardiac or renal failure; such states are always worsening the patient’s condition and may be life-threatening.
Familial Renal Amyloidosis
IgA Nephropathy
Light Chain-Associated Renal Disorders
Nephrotic Syndrome
Diabetic Nephropathy
Heart Failure
Immunoglobulin-Related Amyloidosis
Multiple Myeloma
Treatment of Underlying Plasma Cell Disorder:
Multiple Myeloma or Monoclonal Gammopathy of Undetermined Significance (MGUS):
Chemotherapy: Medications like bortezomib, lenalidomide, and dexamethasone are utilized in the treatment process with the aim of extirpating the aberrant plasma cells that produce the light chains.
Immunomodulatory Agents: Plasma cell dyscrasia could also be treated with the help of agents such as thalidomide and pomalidomide.
Targeted Therapy: ERAD components include proteasome inhibitors (e. g., carfilzomib) and monoclonal antibodies (e. g., daratumumab) that slow down the generation of light chains.
Stem Cell Transplant: Intensification with high-dose chemotherapy and autologous stem cell rescue could be used for intensification for selected patients with a view of inducing further disease remission.
Management of Renal Complications:
Control of Proteinuria: Renal management that includes the treatment with angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) with the purpose of reduction of proteinuria and protection of renal function.
Management of Hypertension B: antihypertensive agents that will help to lower the blood pressure to avoid further deterioration of renal function.
Renal Replacement Therapy: In case of AKI progression to advanced renal impairment or ESRD; dialysis or kidney transplantation may be necessary.
Symptomatic Management:
Edema Management: It should be also mentioned that diuretics are effective in case of fluid overload problem and peripheral edema.
Cardiac Symptoms: Use of cardio-selective drugs such as those that are used in the treatment of congestive heart failure or arrhythmias.
Supportive Care:
Nutritional Support: Low protein and heart healthy diets and nutrition management of renal function and cardiovascular disease.
Pain Management: Pain management following LCDD and its complications; the use of analgesics and other supportive measures.
Hematology
Dietary Modifications:
Protein Intake: A low protein diet is also useful in decreasing kidney workload and in controlling proteinuria.
Sodium and Fluid Restriction: Restriction in salt intake as well as restriction in the intake of fluids reduces hypertension and edema respectively.
Heart-Healthy Diet: A diet restricted in saturated fat, cholesterol, and sodium enhances the cardiovascular health.
Lifestyle Changes:
Weight Management: Control of diet and exercise also plays a crucial role in weight control which is in support of the cardiovascular health and to minimise stress on the kidney.
Exercise: Likelihood of decreased exercise related to cardiovascular health and Chronic illnesses conditions are reduced through, moderate, regular exercise.
Smoking Cessation: Smoking cessation is beneficial in all aspects of health and especially in the cardiovascular health.
Hematology
Bortezomib (Velcade): It is employed to target the proteasome needed in the breakdown of proteins that control cell division and death. This has the effect of lowering the count of malignant plasma cells and diminishes the synthesis of monoclonal light chains.
Carfilzomib (Kyprolis): Like bortezomib, carfilzomib is another second-generation proteasome inhibitor which also aims at the target to decrease the plasma cells.
Hematology
Lenalidomide (Revlimid): Lenalidomide is an anti-angiogenic, anti-inflammatory, and immunomodulating agent. It diminishes the amount of abnormal plasma cells and aids in elevating the immune system functions in relation to killing cancerous cells.
Thalidomide: Thalidomide has relatively fewer applications compared to other drugs because of its side effects, but it has immunomodulatory effects that can assist in managing the proliferation of the malignant plasma cells.
Hematology
Dexamethasone: Dexamethasone being a corticosteroid is sometimes used in conjunction with other agents used for chemotherapy, to minimize inflammation and slowing the proliferation of malignant plasma cells. They are included in the standard therapy protocols for patients with plasma cell diseases.
Hematology
Daratumumab:It is a monoclonal antibody that binds to the CD38 receptor which is present on the surface of plasma cells. It is also useful in decreasing the number of malignant plasma cells and, as a result, the number of light chains produced is reduced.
Hematology
Plasma Exchange: It is performed to eliminate light chains from the bloodstream with the aim of alleviating pathologic manifestations connected to the renal and extrarenal deposits. The plasma with light chains is separated from the patient’s blood and a machine returns the rest of blood components and pumps in plasma or albumin.
Kidney Biopsy: It is used in the diagnosis of LCDD to look for the presence of typical light chains deposits into the kidney tissue. Mostly, a small amount of tissue from the kidney is collected; this process is usually done using a needle inserted into the kidney with the help of images. Light microscopical, immunofluorescent and electron microscopical examinations of the tissue are then performed.
Hematology
Approaches used in light chain deposition disease are broadly categorized into phases. First, the diagnosis and the initial evaluation are necessary and accomplished through biopsy and imaging to assess the involvement of the organ. The treatment phase is aimed to deal with the plasma cell dyrease with the help of chemotherapeutic drugs (bortezomib, lenalidomide), to treat the symptoms such as proteinuria and hypertension and to support with dietary/nutritional and lifestyle interventions. Response monitoring includes constant assessment of the efficacy of the treatment and modifying the therapy if necessary. Finally, patients with end-stage renal disease (ESRD) or cardiovascular problems are treated in the advanced phase of the disease and patient counseling and follow-up are performed. If required, the end-of-life or palliative care is offered, to improve the quality of life.
Light-Chain Deposition Disease (LCDD) is an idiopathic condition associated with the extracellular deposition of monoclonal immunoglobulin light chains (predominantly the kappa type) in organ tissues with the kidney being most affected. It is one of the monoclonal gammopathies which are clinical conditions resulting from the increased production of monoclonal immunoglobulins or their fragments by abnormal plasma cells. It is often linked to a pre-existing plasma cell dyscrasia, including multiple myeloma or monoclonal gammopathy of unknown origin (MGUS). The plasma cells that are affected by the cancer form abnormal light chains that circulate in the blood.
Currently, there is no accurate statistical data on the incidence rates of light-chain deposition disease (LCDD). Though, it is detected in approximately 5% of the patients who have passed away due to multiple myeloma. Major mortality and morbidity factors in LCDD are often associated with kidney-related ailment comprisinghypertension, nephrotic syndrome and progression to ESRD. Also, liver involvement can cause the liver to malfunction and ultimately produce hepatic failure.
Renal LCDD has distinct features at the microscopic level and these include nodular sclerosing glomerulonephritis on LM, diffuse linear light chain deposition on IFM, and electron-dense deposits on EM. It concerns the abnormal accumulation of kappa light chains in the kidneys, within glomerular tufts and tubular basement membranes, which may cause kidney impairment.
Specimens stained for kappa and lambda chains is essential for diagnosis and positivity of kappa chains are often observed along tubular basement membrane. Electron microscopy can be useful in the identification of LCDD from other types of amyloid light-chain (AL) amyloidosis which is mostly associated with lambda light chain.
The involvement of extra renal organs in LCDD is limited but organs which may be involved include the liver, heart, lungs and the peripheral nerves. The liver is another site which is affected in more than 90 percent of patients with diabetic nephropathy and may result in liver cirrhosis and liver failure. Some of the effects of systemic sclerosis on the heart are cardiomyopathy and congestive heart failure and, on the lung, lung disease. Peripheral neuropathy and in some cases even brain involvement has also been observed.
LCDD is most linked to multiple myeloma in patients, where it occurs in approximately 58% of patients. It may be diagnosed at the time of a suspected plasma cell neoplasm or as a manifestation of multiple myeloma at relapse. LCDD may also affect the other subtypes of B-cell lymphomas.
Light-Chain Deposition Disease (LCDD) is primarily related to plasma cell dyscrasias, more commonly multiple myeloma and Monoclonal Gammopathy of Undetermined Significance (MGUS), in which aberrant plasma cells synthesize and release excessive monoclonal light chains that precipitate in tissues. It can also be associated with other B-cell lymphomas, namely, lymphoplasmacytic lymphoma and chronic lymphocytic leukemia. These conditions may be caused in part by genetic susceptibility, but environmental factors, chronic inflammation, and infections also may be involved, although these factors are not as well defined as genetic factors.
For instance, the median survival for patients with light-chain deposition disease (LCDD) is under 4 years. In the largest study to date, patients were followed for a median of 27 months; 57% developed uremia, and 59% had died. Of the predictors, patient age, the diagnosis of multiple myeloma, and extrarenal light-chain deposition. Most important, ESRD and need for dialysis did not adversely affect the prognosis.”
These factors include age, multiple myeloma or cast nephropathy, and the presence of extrarenal light chain deposition.
Given the fact that LCDD is rare, it remains difficult to identify the natural course of the disease or the effects of its treatment.
Age Group
The most common age group for LCDD is adults, and more specifically, those of middle to older age, in their 50s – 70s. It is not common in young persons especially at the early ages.
End-organ dysfunction is frequent in LCDD and may dominate clinical manifestations of the disease in the form of hypertension, peripheral oedema, neuropathy or congestive heart failure. It is understood that approximately 50% of LCDD patients have nephrotic syndrome. However, the proteinuria is less than 1g/day in about 25% of cases because of Tubulointerstitial Involvement. Abnormal physical signs that may be looked for include new heart sounds and splenomegaly.
Multiple myeloma
B-Cell lymphoma
Monoclonal Gammopathy of Undetermined Significance (MGUS)
Congestive heart failure
Gradual Onset: LCDD typically has a gradual clinical course with the development of symptoms during months and years.
Chronic Presentation: Many patients are usually asymptomatic or have chronic complaints like swelling, high BP or slowly progressive renal failure.
Acute Exacerbations: There can be severe exacerbations in some cases, often connected with cardiac or renal failure; such states are always worsening the patient’s condition and may be life-threatening.
Familial Renal Amyloidosis
IgA Nephropathy
Light Chain-Associated Renal Disorders
Nephrotic Syndrome
Diabetic Nephropathy
Heart Failure
Immunoglobulin-Related Amyloidosis
Multiple Myeloma
Treatment of Underlying Plasma Cell Disorder:
Multiple Myeloma or Monoclonal Gammopathy of Undetermined Significance (MGUS):
Chemotherapy: Medications like bortezomib, lenalidomide, and dexamethasone are utilized in the treatment process with the aim of extirpating the aberrant plasma cells that produce the light chains.
Immunomodulatory Agents: Plasma cell dyscrasia could also be treated with the help of agents such as thalidomide and pomalidomide.
Targeted Therapy: ERAD components include proteasome inhibitors (e. g., carfilzomib) and monoclonal antibodies (e. g., daratumumab) that slow down the generation of light chains.
Stem Cell Transplant: Intensification with high-dose chemotherapy and autologous stem cell rescue could be used for intensification for selected patients with a view of inducing further disease remission.
Management of Renal Complications:
Control of Proteinuria: Renal management that includes the treatment with angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) with the purpose of reduction of proteinuria and protection of renal function.
Management of Hypertension B: antihypertensive agents that will help to lower the blood pressure to avoid further deterioration of renal function.
Renal Replacement Therapy: In case of AKI progression to advanced renal impairment or ESRD; dialysis or kidney transplantation may be necessary.
Symptomatic Management:
Edema Management: It should be also mentioned that diuretics are effective in case of fluid overload problem and peripheral edema.
Cardiac Symptoms: Use of cardio-selective drugs such as those that are used in the treatment of congestive heart failure or arrhythmias.
Supportive Care:
Nutritional Support: Low protein and heart healthy diets and nutrition management of renal function and cardiovascular disease.
Pain Management: Pain management following LCDD and its complications; the use of analgesics and other supportive measures.
Hematology
Dietary Modifications:
Protein Intake: A low protein diet is also useful in decreasing kidney workload and in controlling proteinuria.
Sodium and Fluid Restriction: Restriction in salt intake as well as restriction in the intake of fluids reduces hypertension and edema respectively.
Heart-Healthy Diet: A diet restricted in saturated fat, cholesterol, and sodium enhances the cardiovascular health.
Lifestyle Changes:
Weight Management: Control of diet and exercise also plays a crucial role in weight control which is in support of the cardiovascular health and to minimise stress on the kidney.
Exercise: Likelihood of decreased exercise related to cardiovascular health and Chronic illnesses conditions are reduced through, moderate, regular exercise.
Smoking Cessation: Smoking cessation is beneficial in all aspects of health and especially in the cardiovascular health.
Hematology
Bortezomib (Velcade): It is employed to target the proteasome needed in the breakdown of proteins that control cell division and death. This has the effect of lowering the count of malignant plasma cells and diminishes the synthesis of monoclonal light chains.
Carfilzomib (Kyprolis): Like bortezomib, carfilzomib is another second-generation proteasome inhibitor which also aims at the target to decrease the plasma cells.
Hematology
Lenalidomide (Revlimid): Lenalidomide is an anti-angiogenic, anti-inflammatory, and immunomodulating agent. It diminishes the amount of abnormal plasma cells and aids in elevating the immune system functions in relation to killing cancerous cells.
Thalidomide: Thalidomide has relatively fewer applications compared to other drugs because of its side effects, but it has immunomodulatory effects that can assist in managing the proliferation of the malignant plasma cells.
Hematology
Dexamethasone: Dexamethasone being a corticosteroid is sometimes used in conjunction with other agents used for chemotherapy, to minimize inflammation and slowing the proliferation of malignant plasma cells. They are included in the standard therapy protocols for patients with plasma cell diseases.
Hematology
Daratumumab:It is a monoclonal antibody that binds to the CD38 receptor which is present on the surface of plasma cells. It is also useful in decreasing the number of malignant plasma cells and, as a result, the number of light chains produced is reduced.
Hematology
Plasma Exchange: It is performed to eliminate light chains from the bloodstream with the aim of alleviating pathologic manifestations connected to the renal and extrarenal deposits. The plasma with light chains is separated from the patient’s blood and a machine returns the rest of blood components and pumps in plasma or albumin.
Kidney Biopsy: It is used in the diagnosis of LCDD to look for the presence of typical light chains deposits into the kidney tissue. Mostly, a small amount of tissue from the kidney is collected; this process is usually done using a needle inserted into the kidney with the help of images. Light microscopical, immunofluorescent and electron microscopical examinations of the tissue are then performed.
Hematology
Approaches used in light chain deposition disease are broadly categorized into phases. First, the diagnosis and the initial evaluation are necessary and accomplished through biopsy and imaging to assess the involvement of the organ. The treatment phase is aimed to deal with the plasma cell dyrease with the help of chemotherapeutic drugs (bortezomib, lenalidomide), to treat the symptoms such as proteinuria and hypertension and to support with dietary/nutritional and lifestyle interventions. Response monitoring includes constant assessment of the efficacy of the treatment and modifying the therapy if necessary. Finally, patients with end-stage renal disease (ESRD) or cardiovascular problems are treated in the advanced phase of the disease and patient counseling and follow-up are performed. If required, the end-of-life or palliative care is offered, to improve the quality of life.
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