Immunoglobulin Light Chains
Genes, Immunoglobulin Light Chain
Gene Rearrangement, B-Lymphocyte, Light Chain
Bence Jones Protein
Immunoglobulin Variable Region
Immunoglobulin Constant Regions
Immunoglobulin Light Chains, Surrogate
Immunoglobulin Heavy Chains
Myosin Light Chains
Amino Acid Sequence
Immunoglobulin Joining Region
Molecular Sequence Data
Gene Rearrangement, B-Lymphocyte
Receptors, Antigen, B-Cell
Polymerase Chain Reaction
Binding Sites, Antibody
Complementarity Determining Regions
Nephelometry and Turbidimetry
Electrophoresis, Polyacrylamide Gel
Leukemia, Lymphocytic, Chronic, B-Cell
Nucleic Acid Hybridization
Immunoglobulin A, Secretory
Sequence Homology, Nucleic Acid
Sequence Homology, Amino Acid
Immunoglobulin Fab Fragments
Immunoglobulin Fc Fragments
Immunoglobulin Class Switching
Induction of Ig light chain gene rearrangement in heavy chain-deficient B cells by activated Ras. (1/1652)During B cell development, rearrangement and expression of Ig heavy chain (HC) genes promote development and expansion of pre-B cells accompanied by the onset of Ig light chain (LC) variable region gene assembly. To elucidate the signaling pathways that control these events, we have tested the ability of activated Ras expression to promote B cell differentiation to the stage of LC gene rearrangement in the absence of Ig HC gene expression. For this purpose, we introduced an activated Ras expression construct into JH-deleted embryonic stem cells that lack the ability to assemble HC variable region genes and assayed differentiation potential by recombination activating gene (RAG) 2-deficient blastocyst complementation. We found that activated Ras expression induces the progression of B lineage cells beyond the developmental checkpoint ordinarily controlled by mu HC. Such Ras/JH-deleted B cells accumulate in the periphery but continue to express markers associated with precursor B cells including RAG gene products. These peripheral Ras/JH-deleted B cell populations show extensive Ig LC gene rearrangement but maintain an extent of kappa LC gene rearrangement and a preference for kappa over lambda LC gene rearrangement similar to that of wild-type B cells. We discuss these findings in the context of potential mechanisms that may regulate Ig LC gene rearrangement. (+info)
Characterization of an immunoglobin cDNA clone containing the variable and constant regions for the MOPC 21 kappa light chain. (2/1652)Nucleotide sequence analysis and restriction endonuclease mapping have been used to characterize a cDNA copy of immunoglobulin MOPC 21 Kappa mRNA clones in the bacterial plasmid pMB9. Three regions of the inserted cDNA of plasmid pL21-1 have been sequenced and match the known protein sequence at amino acid residues 1-24, 128-138 and 171-179. With these sequences to provide absolute correlations between the restriction map and the structural gene sequence it has been possible to exactly deduce the positions of all 11 of the insert restriction sites mapped within the structural gene. The pL21-1 insert contains the complete variable and constant regions as well as parts of the 3' untranslated and polypeptide leader coding sequences. (+info)
Recombinant DNA clones constructed from immunoglobulin kappa light chain messenger RNA. (3/1652)Recombinant DNA clones have been generated from mouse myeloma MOPC 21 immunoglobulin kappa light chain mRNA. Complementary DNA (cDNA) synthesized on kappa light chain mRNA by reverse transcriptase was made double stranded and inserted into the bacterial plasmid vector, pMB9. Approximately 70 tetracycline-resistant transformed colonies containing kappa light chain mRNA sequences were identified by colony hybridization. Five of these recombinant clones were selected and characterized. Three clones contain both kappa light chain constant and variable region sequences. Two of these three recombinant clones have been shown to include all of the kappa light chain constant and variable region coding sequences. Another of the five selected recombinant clones contain kappa light chain constant region sequences. The remaining characterized clone appears to be derived from sequences at the 5'-end of kappa light chain mRNA, possibly extending to the terminal cap structure. (+info)
Novel mechanisms control the folding and assembly of lambda5/14.1 and VpreB to produce an intact surrogate light chain. (4/1652)Surrogate light chain, which escorts the mu heavy chain to the cell surface, is a critical component of the pre-B cell receptor complex. The two proteins that comprise the surrogate light chain, VpreB and lambda5/14.1, contain both unique regions and Ig-like domains. The unique regions have been postulated to function in the assembly of the surrogate light chain. However, by using transient transfection of COS7 cells, we show that deletion of the unique regions of both proteins did not inhibit the assembly of surrogate light chain. Instead, in vivo folding studies showed that the unique region of lambda5/14.1 acts as an intramolecular chaperone by preventing the folding of this protein when it is expressed in the absence of its partner, VpreB. The Ig domains of both lambda5/14.1 and VpreB are atypical. The one in VpreB lacks one of the canonical beta strands whereas the one in lambda5/14.1 has an extra beta strand. Deletion of the extra beta strand in lambda5/14.1 completely abrogated the formation of the surrogate light chain, demonstrating that complementation of the incomplete Ig domain in VpreB by the extra beta strand in lambda5/14.1 was necessary and sufficient for the folding and assembly of these proteins. Our studies reveal two novel mechanisms for regulating surrogate light chain formation: (i) the presence of an intramolecular chaperone that prevents folding of the unassembled subunit but that remains part of the mature assembled protein, and (ii) splitting an Ig domain between two proteins to control their folding and assembly. (+info)
Assignment of genes for immunoglobulin kappa and heavy chains to chromosomes 6 and 12 in mouse. (5/1652)Using somatic cell hybrids from fusions of lymphocytes of two different mouse stocks with the myeloma cell line X63-Ag8, we have assigned genes for the immunoglobulin heavy and kappa-type light chains to chromosomes 12 and 6, respectively. The two mouse stocks exhibit karyotypes consisting of nine pairs of metacentric chromosomes as a result of centric fusions of acrocentric chromosomes in different combinations. In the hybrid cells these metacentric chromosomes can be distinguished from the acrocentric chromosomes of myeloma origin, permitting correlation of Ig chain expression with mitotic loss of individual metacentric chromosomes. (+info)
The structure of an entire noncovalent immunoglobulin kappa light-chain dimer (Bence-Jones protein) reveals a weak and unusual constant domains association. (6/1652)Monoclonal free light chains secreted in immunoproliferative disorders are frequently involved in renal complications, including a specific proximal tubule impairment, Fanconi's syndrome. The latter is characterized in most cases by intracellular crystallization including a light-chain variable-domain fragment which resists lysosomal proteases. Bence-Jones protein (BJP) DEL was isolated from a patient with myeloma-associated Fanconi's syndrome. The crystal structure of this human kappa immunoglobulin light-chain noncovalent dimer was determined using molecular replacement with the structure of molecule REI, as the variable domain, and that of BJP LOC as the constant domain. To our knowledge, DEL is the first complete kappa BJP structure described to date. The R-factor is 20.7% at 2.8 A resolution. The BJP DEL dimer was compared with other light-chain dimers and with Fab fragments with a kappa light chain. Although the domain-folding pattern was similar, the relative positions of the constant domains differed. BJP DEL showed a noncanonical quaternary structural arrangement which may be attributable to the poor CL-CL affinity and lack of an interchain disulfide bridge, combined with the conformational editing effect of the crystal-packing forces. Our results suggest that, in the absence of a disulfide bridge, most BJP CLs are probably mobile in solution. This may explain their high susceptibility to proteases and the absence of naturally occurring crystals for these dimers. Furthermore, these findings of an unusual quaternary structure of an immunoglobulin light-chain association extend our knowledge about the large and highly diverse structures of the immunoglobulin superfamily. (+info)
Physicochemical consequences of amino acid variations that contribute to fibril formation by immunoglobulin light chains. (7/1652)The most common form of systemic amyloidosis originates from antibody light chains. The large number of amino acid variations that distinguish amyloidogenic from nonamyloidogenic light chain proteins has impeded our understanding of the structural basis of light-chain fibril formation. Moreover, even among the subset of human light chains that are amyloidogenic, many primary structure differences are found. We compared the thermodynamic stabilities of two recombinant kappa4 light-chain variable domains (V(L)s) derived from amyloidogenic light chains with a V(L) from a benign light chain. The amyloidogenic V(L)s were significantly less stable than the benign V(L). Furthermore, only the amyloidogenic V(L)s formed fibrils under native conditions in an in vitro fibril formation assay. We used site-directed mutagenesis to examine the consequences of individual amino acid substitutions found in the amyloidogenic V(L)s on stability and fibril formation capability. Both stabilizing and destabilizing mutations were found; however, only destabilizing mutations induced fibril formation in vitro. We found that fibril formation by the benign V(L) could be induced by low concentrations of a denaturant. This indicates that there are no structural or sequence-specific features of the benign V(L) that are incompatible with fibril formation, other than its greater stability. These studies demonstrate that the V(L) beta-domain structure is vulnerable to destabilizing mutations at a number of sites, including complementarity determining regions (CDRs), and that loss of variable domain stability is a major driving force in fibril formation. (+info)
Cells with clonal light chains are present in peripheral blood at diagnosis and in apheretic stem cell harvests of primary amyloidosis. (8/1652)In primary systemic amyloidosis, small numbers of bone marrow plasma cells secrete monoclonal light chains that form extracellular fibrils (amyloid) in various organs. Evidence limited to a few cases suggests that rare clonal elements can also be found in the peripheral blood (PB), and this may be relevant in PB stem cell autotransplantation. Since up to 40% of amyloid clones do not synthesize heavy chains, in order to detect tumor cells with high specificity and sensitivity we developed a seminested allele-specific oligonucleotide polymerase chain reaction for tumor light chains. Clone-related sequences were detected in DNA and/or cDNA from the PB cells of eight of 10 patients at diagnosis and from apheretic collections of three of four cases undergoing PB progenitor autotransplantation. Since there are experimental data suggesting that circulating tumor cells may be involved in the growth of the amyloidogenic clone and may be chemoresistant, these findings are relevant to the use of leukapheresis purging strategies for PB progenitor autotransplantation in amyloidosis. (+info)
There are several types of amyloidosis, each with different causes and symptoms. The most common types include:
1. Primary amyloidosis: This type is caused by the production of abnormal proteins in the bone marrow. It mainly affects older adults and can lead to symptoms such as fatigue, weight loss, and numbness or tingling in the hands and feet.
2. Secondary amyloidosis: This type is caused by other conditions, such as rheumatoid arthritis, tuberculosis, or inflammatory bowel disease. It can also be caused by long-term use of certain medications, such as antibiotics or chemotherapy.
3. Familial amyloid polyneuropathy: This type is inherited and affects the nerves in the body, leading to symptoms such as muscle weakness, numbness, and pain.
4. Localized amyloidosis: This type affects a specific area of the body, such as the tongue or the skin.
The symptoms of amyloidosis can vary depending on the organs affected and the severity of the condition. Some common symptoms include:
4. Numbness or tingling in the hands and feet
5. Swelling in the legs, ankles, and feet
6. Difficulty with speech or swallowing
8. Heart problems
9. Kidney failure
10. Liver failure
The diagnosis of amyloidosis is based on a combination of physical examination, medical history, laboratory tests, and imaging studies. Laboratory tests may include blood tests to measure the levels of certain proteins in the body, as well as biopsies to examine tissue samples under a microscope. Imaging studies, such as X-rays, CT scans, and MRI scans, may be used to evaluate the organs affected by the condition.
There is no cure for amyloidosis, but treatment can help manage the symptoms and slow the progression of the disease. Treatment options may include:
1. Medications to control symptoms such as pain, swelling, and heart problems
2. Chemotherapy to reduce the production of abnormal proteins
3. Autologous stem cell transplantation to replace damaged cells with healthy ones
4. Dialysis to remove excess fluids and waste products from the body
5. Nutritional support to ensure adequate nutrition and hydration
6. Physical therapy to maintain muscle strength and mobility
7. Supportive care to manage pain, improve quality of life, and reduce stress on the family.
In conclusion, amyloidosis is a complex and rare group of diseases that can affect multiple organs and systems in the body. Early diagnosis and treatment are essential to managing the symptoms and slowing the progression of the disease. It is important for patients with suspected amyloidosis to seek medical attention from a specialist, such as a hematologist or nephrologist, for proper evaluation and treatment.
Plasmacytoma is a type of plasma cell dyscrasia, which is a group of diseases that affect the production and function of plasma cells. Plasma cells are a type of white blood cell that produces antibodies to fight infections. In plasmacytoma, the abnormal plasma cells grow and multiply out of control, leading to a tumor.
There are several subtypes of plasmacytoma, including:
* solitary plasmacytoma: A single tumor that occurs in one location.
* multiple myeloma: A type of cancer that affects the bones and is characterized by an overgrowth of malignant plasma cells in the bone marrow.
* extramedullary plasmacytoma: A tumor that occurs outside of the bone marrow, such as in soft tissue or organs.
Plasmacytoma is usually diagnosed through a combination of physical examination, imaging tests such as X-rays or CT scans, and biopsy. Treatment typically involves chemotherapy and/or radiation therapy to destroy the abnormal cells. In some cases, surgery may be necessary to remove the tumor.
Plasmacytoma is a relatively rare cancer, but it can be aggressive and potentially life-threatening if left untreated. It is important for patients with symptoms of plasmacytoma to seek medical attention as soon as possible to receive an accurate diagnosis and appropriate treatment.
Multiple myeloma is the second most common type of hematologic cancer after non-Hodgkin's lymphoma, accounting for approximately 1% of all cancer deaths worldwide. It is more common in older adults, with most patients being diagnosed over the age of 65.
The exact cause of multiple myeloma is not known, but it is believed to be linked to genetic mutations that occur in the plasma cells. There are several risk factors that have been associated with an increased risk of developing multiple myeloma, including:
1. Family history: Having a family history of multiple myeloma or other plasma cell disorders increases the risk of developing the disease.
2. Age: The risk of developing multiple myeloma increases with age, with most patients being diagnosed over the age of 65.
3. Race: African Americans are at higher risk of developing multiple myeloma than other races.
4. Obesity: Being overweight or obese may increase the risk of developing multiple myeloma.
5. Exposure to certain chemicals: Exposure to certain chemicals such as pesticides, solvents, and heavy metals has been linked to an increased risk of developing multiple myeloma.
The symptoms of multiple myeloma can vary depending on the severity of the disease and the organs affected. Common symptoms include:
1. Bone pain: Pain in the bones, particularly in the spine, ribs, or long bones, is a common symptom of multiple myeloma.
2. Fatigue: Feeling tired or weak is another common symptom of the disease.
3. Infections: Patients with multiple myeloma may be more susceptible to infections due to the impaired functioning of their immune system.
4. Bone fractures: Weakened bones can lead to an increased risk of fractures, particularly in the spine, hips, or ribs.
5. Kidney problems: Multiple myeloma can cause damage to the kidneys, leading to problems such as kidney failure or proteinuria (excess protein in the urine).
6. Anemia: A low red blood cell count can cause anemia, which can lead to fatigue, weakness, and shortness of breath.
7. Increased calcium levels: High levels of calcium in the blood can cause symptoms such as nausea, vomiting, constipation, and confusion.
8. Neurological problems: Multiple myeloma can cause neurological problems such as headaches, numbness or tingling in the arms and legs, and difficulty with coordination and balance.
The diagnosis of multiple myeloma typically involves a combination of physical examination, medical history, and laboratory tests. These may include:
1. Complete blood count (CBC): A CBC can help identify abnormalities in the numbers and characteristics of different types of blood cells, including red blood cells, white blood cells, and platelets.
2. Serum protein electrophoresis (SPEP): This test measures the levels of different proteins in the blood, including immunoglobulins (antibodies) and abnormal proteins produced by myeloma cells.
3. Urine protein electrophoresis (UPEP): This test measures the levels of different proteins in the urine.
4. Immunofixation: This test is used to identify the type of antibody produced by myeloma cells and to rule out other conditions that may cause similar symptoms.
5. Bone marrow biopsy: A bone marrow biopsy involves removing a sample of tissue from the bone marrow for examination under a microscope. This can help confirm the diagnosis of multiple myeloma and determine the extent of the disease.
6. Imaging tests: Imaging tests such as X-rays, CT scans, or MRI scans may be used to assess the extent of bone damage or other complications of multiple myeloma.
7. Genetic testing: Genetic testing may be used to identify specific genetic abnormalities that are associated with multiple myeloma and to monitor the response of the disease to treatment.
It's important to note that not all patients with MGUS or smoldering myeloma will develop multiple myeloma, and some patients with multiple myeloma may not have any symptoms at all. However, if you are experiencing any of the symptoms listed above or have a family history of multiple myeloma, it's important to talk to your doctor about your risk and any tests that may be appropriate for you.
There are several types of paraproteinemias, including:
1. Multiple myeloma: This is a type of cancer that affects the plasma cells in the bone marrow, leading to an overproduction of immunoglobulins.
2. Monoclonal gammopathy of undetermined significance (MGUS): This is a condition in which there is an abnormal increase in the level of immunoglobulins in the blood, but the cause cannot be determined.
3. Waldenström macroglobulinemia: This is a rare type of cancer that affects the plasma cells in the bone marrow and leads to an overproduction of immunoglobulins.
4. Primary amyloidosis: This is a condition in which abnormal proteins called amyloids accumulate in the organs, leading to damage and dysfunction.
5. Secondary amyloidosis: This is a condition in which abnormal proteins called amyloids accumulate in the organs due to another underlying condition, such as rheumatoid arthritis or systemic lupus erythematosus.
The symptoms of paraproteinemias can vary depending on the type and severity of the disorder. Common symptoms include fatigue, weakness, weight loss, infections, kidney damage, and bone pain. Treatment options for paraproteinemias depend on the specific type of disorder and may include chemotherapy, radiation therapy, or medications to reduce protein production.
There are several different types of pseudolymphoma, including:
1. Cutaneous pseudolymphoma: This type of pseudolymphoma affects the skin and can appear as a rash or lump.
2. Nodular pseudolymphoma: This type of pseudolymphoma affects the lymph nodes and can cause swelling in the neck, armpits, or groin.
3. Extranodal pseudolymphoma: This type of pseudolymphoma can occur outside of the lymph nodes and can affect other organs, such as the spleen or liver.
4. Lymphomatoid papulosis: This is a rare type of pseudolymphoma that can cause recurring episodes of swelling in the lymph nodes or skin.
The exact cause of pseudolymphoma is not known, but it is thought to be related to an abnormal response of the immune system. Treatment for pseudolymphoma typically involves surgery to remove the affected tissue, as well as radiation therapy and chemotherapy to kill any remaining cancer cells. In some cases, the condition may go into remission on its own without treatment.
The disease is named after the Swedish physician Jan G. Waldenström, who first described it in 1944. It is also known as lymphoplasmacytic lymphoma or IgM multoculullarity.
The exact cause of Waldenström macroglobulinemia is not known, but it is believed to be linked to genetic mutations that occur in the plasma cells. The condition usually affects older adults and is more common in males than females.
Symptoms of Waldenström macroglobulinemia can include:
* Weight loss
* Enlargement of the liver and spleen
* Swelling in the legs, ankles, and hands
* Pain in the bones or joints
* Increased risk of infections
* Numbness or tingling in the hands and feet
The diagnosis of Waldenström macroglobulinemia is based on a combination of physical examination, blood tests, and imaging studies. Treatment options include chemotherapy, immunomodulatory drugs, and stem cell transplantation. The prognosis for the disease varies depending on the severity of the symptoms and the response to treatment.
Overall, Waldenström macroglobulinemia is a rare and complex condition that requires careful management by a team of healthcare professionals. With appropriate treatment, many patients with this condition can experience long-term remission and improved quality of life.
The symptoms of Fanconi Syndrome can vary in severity and may include:
* Abdominal pain
* Failure to gain weight or grow at the expected rate
* Increased risk of infections
* Poor blood sugar control
* High levels of amino acids in the urine
* Abnormal kidney function
Fanconi Syndrome is usually diagnosed through a combination of clinical evaluation, laboratory tests, and genetic analysis. Treatment for the condition typically involves managing the symptoms and addressing any underlying complications. This may include:
* Nutritional support to ensure adequate intake of essential nutrients
* Hydration to prevent dehydration
* Antibiotics to prevent or treat infections
* Medications to manage diarrhea and abdominal pain
* Monitoring of blood sugar levels
* Kidney function tests to monitor for any kidney damage
There is no cure for Fanconi Syndrome, but with proper management, individuals with the condition can lead relatively normal lives. It is important for individuals with Fanconi Syndrome to receive regular medical care and follow a carefully planned diet to manage their symptoms and prevent complications.
There are several causes of hypergammaglobulinemia, including:
1. Chronic infections: Prolonged infections can cause an increase in the production of immunoglobulins to fight off the infection.
2. Autoimmune disorders: Conditions such as rheumatoid arthritis, lupus, and multiple sclerosis can cause the immune system to produce excessive amounts of antibodies.
3. Cancer: Some types of cancer, such as Hodgkin's disease and non-Hodgkin's lymphoma, can cause an increase in immunoglobulin production.
4. Genetic disorders: Certain genetic conditions, such as X-linked agammaglobulinemia, can lead to a deficiency or excess of immunoglobulins.
5. Medications: Certain medications, such as corticosteroids and chemotherapy drugs, can suppress the immune system and reduce the production of immunoglobulins.
Symptoms of hypergammaglobulinemia can include:
1. Infections: Recurring infections are a common symptom of hypergammaglobulinemia, as the excessive amount of antibodies can make it difficult for the body to fight off infections effectively.
2. Fatigue: Chronic infections and inflammation can cause fatigue and weakness.
3. Weight loss: Recurring infections and chronic inflammation can lead to weight loss and malnutrition.
4. Swollen lymph nodes: Enlarged lymph nodes are a common symptom of hypergammaglobulinemia, as the body tries to fight off infections.
5. Fever: Recurring fevers can be a symptom of hypergammaglobulinemia, as the body tries to fight off infections.
6. Night sweats: Excessive sweating at night can be a symptom of hypergammaglobulinemia.
7. Skin rashes: Certain types of skin rashes can be a symptom of hypergammaglobulinemia, such as a rash caused by allergic reactions to medications or infections.
8. Joint pain: Pain and stiffness in the joints can be a symptom of hypergammaglobulinemia, particularly if the excessive amount of antibodies is causing inflammation in the joints.
9. Headaches: Chronic headaches can be a symptom of hypergammaglobulinemia, particularly if the excessive amount of antibodies is causing inflammation in the brain or other parts of the body.
10. Swollen liver and spleen: Enlarged liver and spleen can be a symptom of hypergammaglobulinemia, as the body tries to filter out excess antibodies and fight off infections.
It is important to note that these symptoms can also be caused by other medical conditions, so it is essential to consult a healthcare professional for proper diagnosis and treatment. A healthcare professional may perform blood tests and other diagnostic procedures to determine the underlying cause of the symptoms and develop an appropriate treatment plan. Treatment for hypergammaglobulinemia typically involves addressing the underlying cause of the condition, such as infections, allergies, or autoimmune disorders, and may include medications to reduce inflammation and suppress the immune system.
There are several subtypes of lymphoma, B-cell, including:
1. Diffuse large B-cell lymphoma (DLBCL): This is the most common type of B-cell lymphoma and typically affects older adults.
2. Follicular lymphoma: This type of lymphoma grows slowly and often does not require treatment for several years.
3. Marginal zone lymphoma: This type of lymphoma develops in the marginal zone of the spleen or other lymphoid tissues.
4. Hodgkin lymphoma: This is a type of B-cell lymphoma that is characterized by the presence of Reed-Sternberg cells, which are abnormal cells that can be identified under a microscope.
The symptoms of lymphoma, B-cell can vary depending on the subtype and the location of the tumor. Common symptoms include swollen lymph nodes, fatigue, fever, night sweats, and weight loss.
Treatment for lymphoma, B-cell usually involves chemotherapy, which is a type of cancer treatment that uses drugs to kill cancer cells. Radiation therapy may also be used in some cases. In some cases, bone marrow or stem cell transplantation may be recommended.
Prognosis for lymphoma, B-cell depends on the subtype and the stage of the disease at the time of diagnosis. In general, the prognosis is good for patients with early-stage disease, but the cancer can be more difficult to treat if it has spread to other parts of the body.
Prevention of lymphoma, B-cell is not possible, as the exact cause of the disease is not known. However, avoiding exposure to certain risk factors, such as viral infections and pesticides, may help reduce the risk of developing the disease. Early detection and treatment can also improve outcomes for patients with lymphoma, B-cell.
Lymphoma, B-cell is a type of cancer that affects the immune system and can be treated with chemotherapy and other therapies. The prognosis varies depending on the subtype and stage of the disease at diagnosis. Prevention is not possible, but early detection and treatment can improve outcomes for patients with this condition.
There are several types of lymphoma, including:
1. Hodgkin lymphoma: This is a type of lymphoma that originates in the white blood cells called Reed-Sternberg cells. It is characterized by the presence of giant cells with multiple nucleoli.
2. Non-Hodgkin lymphoma (NHL): This is a type of lymphoma that does not meet the criteria for Hodgkin lymphoma. There are many subtypes of NHL, each with its own unique characteristics and behaviors.
3. Cutaneous lymphoma: This type of lymphoma affects the skin and can take several forms, including cutaneous B-cell lymphoma and cutaneous T-cell lymphoma.
4. Primary central nervous system (CNS) lymphoma: This is a rare type of lymphoma that develops in the brain or spinal cord.
5. Post-transplantation lymphoproliferative disorder (PTLD): This is a type of lymphoma that develops in people who have undergone an organ transplant, often as a result of immunosuppressive therapy.
The symptoms of lymphoma can vary depending on the type and location of the cancer. Some common symptoms include:
* Swollen lymph nodes
* Weight loss
* Night sweats
Lymphoma is diagnosed through a combination of physical examination, imaging tests (such as CT scans or PET scans), and biopsies. Treatment options for lymphoma depend on the type and stage of the cancer, and may include chemotherapy, radiation therapy, immunotherapy, or stem cell transplantation.
Overall, lymphoma is a complex and diverse group of cancers that can affect people of all ages and backgrounds. While it can be challenging to diagnose and treat, advances in medical technology and research have improved the outlook for many patients with lymphoma.
In LLCB, the B cells undergo a mutation that causes them to become cancerous and multiply rapidly. This can lead to an overproduction of these cells in the bone marrow, causing the bone marrow to become crowded and unable to produce healthy red blood cells, platelets, and white blood cells.
LLCB is typically a slow-growing cancer, and it can take years for symptoms to develop. However, as the cancer progresses, it can lead to a range of symptoms including fatigue, weakness, weight loss, fever, night sweats, and swollen lymph nodes.
LLCB is typically diagnosed through a combination of physical examination, blood tests, bone marrow biopsy, and imaging studies such as X-rays or CT scans. Treatment options for LLCB include chemotherapy, radiation therapy, and in some cases, stem cell transplantation.
Overall, while LLCB is a serious condition, it is typically slow-growing and can be managed with appropriate treatment. With current treatments, many people with LLCB can achieve long-term remission and a good quality of life.
The different types of familial amyloidosis include:
1. Familial amyloid polyneuropathy (FAP): This is the most common type of familial amyloidosis and is characterized by the accumulation of amyloid fibers in the nerves, leading to progressive nerve damage and loss of sensation.
2. Familial amyloid cardiomyopathy (FAC): This type of amyloidosis affects the heart and is characterized by the accumulation of amyloid fibers in the heart muscle, leading to progressive heart failure.
3. Familial amyloidotic polyneuropathy (FAP): This type of amyloidosis affects the nerves and is characterized by the accumulation of amyloid fibers in the nerves, leading to progressive nerve damage and loss of sensation.
4. Primary amyloidosis (AL): This is a type of amyloidosis that is not inherited and is characterized by the accumulation of amyloid fibers in various organs and tissues throughout the body.
The symptoms of familial amyloidosis can vary depending on the specific type and the organs affected. Common symptoms include:
* Nerve damage and loss of sensation
* Heart failure
* Weakness and fatigue
* Nausea and vomiting
* Weight loss
The diagnosis of familial amyloidosis is based on a combination of clinical findings, laboratory tests, and genetic analysis. Laboratory tests may include:
* Blood tests to measure the level of amyloid fibers in the blood
* Urine tests to measure the level of amyloid fibers in the urine
* Imaging studies such as X-rays, CT scans, or MRI scans to visualize the accumulation of amyloid fibers in the organs and tissues.
Treatment for familial amyloidosis is aimed at managing the symptoms and slowing the progression of the disease. Treatment options may include:
* Medications to manage pain, nausea, and vomiting
* Physical therapy to maintain muscle strength and mobility
* Dietary modifications to manage weight loss and malnutrition
* Heart failure medications to manage heart failure
* Kidney dialysis or transplantation to manage kidney failure
* Stem cell transplantation to slow the progression of the disease.
The prognosis for familial amyloidosis is generally poor, and the disease can be fatal within a few years after diagnosis. However, with early diagnosis and appropriate treatment, some people with familial amyloidosis may experience a better quality of life and longer survival time. It is important to note that there is currently no cure for familial amyloidosis, and research is ongoing to develop new and more effective treatments for the disease.
Types of Kidney Diseases:
1. Acute Kidney Injury (AKI): A sudden and reversible loss of kidney function that can be caused by a variety of factors, such as injury, infection, or medication.
2. Chronic Kidney Disease (CKD): A gradual and irreversible loss of kidney function that can lead to end-stage renal disease (ESRD).
3. End-Stage Renal Disease (ESRD): A severe and irreversible form of CKD that requires dialysis or a kidney transplant.
4. Glomerulonephritis: An inflammation of the glomeruli, the tiny blood vessels in the kidneys that filter waste products.
5. Interstitial Nephritis: An inflammation of the tissue between the tubules and blood vessels in the kidneys.
6. Kidney Stone Disease: A condition where small, hard mineral deposits form in the kidneys and can cause pain, bleeding, and other complications.
7. Pyelonephritis: An infection of the kidneys that can cause inflammation, damage to the tissues, and scarring.
8. Renal Cell Carcinoma: A type of cancer that originates in the cells of the kidney.
9. Hemolytic Uremic Syndrome (HUS): A condition where the immune system attacks the platelets and red blood cells, leading to anemia, low platelet count, and damage to the kidneys.
Symptoms of Kidney Diseases:
1. Blood in urine or hematuria
2. Proteinuria (excess protein in urine)
3. Reduced kidney function or renal insufficiency
4. Swelling in the legs, ankles, and feet (edema)
5. Fatigue and weakness
6. Nausea and vomiting
7. Abdominal pain
8. Frequent urination or polyuria
9. Increased thirst and drinking (polydipsia)
10. Weight loss
Diagnosis of Kidney Diseases:
1. Physical examination
2. Medical history
3. Urinalysis (test of urine)
4. Blood tests (e.g., creatinine, urea, electrolytes)
5. Imaging studies (e.g., X-rays, CT scans, ultrasound)
6. Kidney biopsy
7. Other specialized tests (e.g., 24-hour urinary protein collection, kidney function tests)
Treatment of Kidney Diseases:
1. Medications (e.g., diuretics, blood pressure medication, antibiotics)
2. Diet and lifestyle changes (e.g., low salt intake, increased water intake, physical activity)
3. Dialysis (filtering waste products from the blood when the kidneys are not functioning properly)
4. Kidney transplantation ( replacing a diseased kidney with a healthy one)
5. Other specialized treatments (e.g., plasmapheresis, hemodialysis)
Prevention of Kidney Diseases:
1. Maintaining a healthy diet and lifestyle
2. Monitoring blood pressure and blood sugar levels
3. Avoiding harmful substances (e.g., tobacco, excessive alcohol consumption)
4. Managing underlying medical conditions (e.g., diabetes, high blood pressure)
5. Getting regular check-ups and screenings
Early detection and treatment of kidney diseases can help prevent or slow the progression of the disease, reducing the risk of complications and improving quality of life. It is important to be aware of the signs and symptoms of kidney diseases and seek medical attention if they are present.
Immunoglobulin light chain
Immunoglobulin heavy chain
Signal recognition particle
Serum free light-chain measurement
Bence Jones protein
Large B-cell lymphoma arising in HHV8-associated multicentric Castleman's disease
Splenic marginal zone lymphoma
Cellular adoptive immunotherapy
Paul David Gottlieb
Organization and expression of immunoglobulin genes
Immunoglobulin V-set domain
DNA polymerase lambda
DNA polymerase mu
Index of biochemistry articles
Diffuse proliferative nephritis
Chronic lymphocytic leukemia
Acute lymphoblastic leukemia
List of OMIM disorder codes
Tumors of the hematopoietic and lymphoid tissues
Myosin binding protein C, cardiac
List of MeSH codes (D12.776)
Browsing by Subject "Immunoglobulin Light Chains"
SCOPe 2.03: Protein: Immunoglobulin light chain kappa constant domain, CL-kappa
How to manage Waldenstrom's macroglobulinemia | Leukemia
Biosynthesis of immunoglobulins: the separate classes of polyribosomes synthesizing heavy and light chains - Wikidata
Amyloidosis: Definition of Amyloid and Amyloidosis, Classification Systems, Systemic Amyloidoses
Immunoglobulin analysis tool: A novel tool for the analysis of human and mouse heavy and light chain transcripts - Fingerprint ...
3HC0: Bha10 Igg1 Wild-Type Fab - Antibody Directed At Human Ltbr
Quantitative Bence-Jones protein test: MedlinePlus Medical Encyclopedia
Search Results - The ASCO Post
Notes from the Field: Evaluation of a Perceived Cluster of Plasma Cell Dyscrasias Among Workers at a Natural Gas Company -...
POEMS Syndrome: Still an Enigma?
Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis) Medication: Corticosteroids, Antineoplastics,...
Cy5-Donkey Anti-Rat IgG (H+L) (min X) Secondary Antibody | JIR
Frances Arters | Medicine
NIOSHTIC-2 Search Results - Full View
A Study of JNJ-68284528, a Chimeric Antigen Receptor T Cell (CAR-T) Therapy Directed Against B-cell Maturation Antigen (BCMA)...
NHANES 1988-1994: Monoclonal gammopathy of undetermined significance (MGUS) (Surplus) Data Documentation, Codebook, and...
Goat Anti Rabbit (IgG) secondary antibody preadsorbed Alexa Fluor® 750
FITC Donkey Anti-Rabbit IgG(H+L) | SouthernBiotech
Sebastian Doniach's Profile | Stanford Profiles
WHO EMRO | Review: Multiple myeloma of the central nervous system: a clinicopathological review | Volume 15, issue 6 | EMHJ...
Enzyme-Linked Immunosorbent Assay and Serologic Responses to Pneumocystis jiroveci - Volume 10, Number 5-May 2004 - Emerging...
Cancers | Free Full-Text | Serine Biosynthesis Pathway Supports MYC-miR-494-EZH2 Feed-Forward Circuit Necessary to Maintain...
Goat Anti-Mouse IgG (H+L) Antibody, Polyclonal
SMART: IGc2 domain annotation
NEW (2008) DeCS DESCRIPTORS WITH SCOPE NOTES (UNIT RECORD FORMAT; 21/02/2008
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Drug-drug Interaction and Drug-drug Interaction Evidence Ontology</obo:IAO...
- Diagnoses included MGUS (five persons), myeloma (four), WM (three), and immunoglobulin light chain amyloidosis (one). (cdc.gov)
- The mechanism of toxicity can be aggregation and/or deposit (light-chain amyloidosis, monoclonal immunoglobulin deposition disease, crystal-storing histiocytosis, and type I cryoglobulinemia) or auto-antibody activity (chronic cold agglutinin disease, mixed cryoglobulinemia, xanthomatosis, and demyelinating peripheral neuropathies). (cancernetwork.com)
- Exploiting endogenous and therapy-induced apoptotic vulnerabilities in immunoglobulin light chain amyloidosis with BH3 mimetics. (bu.edu)
- Hughes DM, Henshaw L, Blevins F, Edwards C, Lerner A, Sloan JM, Sanchorawala V. Standard 30-minute Monitoring Time and Less Intensive Pre-medications is Safe in Patients Treated With Subcutaneous Daratumumab for Multiple Myeloma and Light Chain Amyloidosis. (bu.edu)
- Background: Primary systemic (AL) amyloidosis is a rare plasma cell disorder characterized by soft-tissue deposition of monoclonal light chain fragments. (elsevier.com)
- In patient 9, light-chain and pre-albumin (transthyretin) antibodies were related to arthritis and senile amyloidosis, respectively. (opendentistryjournal.com)
- Recurrent mutations of MAPK pathway genes in multiple myeloma but not in amyloid light-chain amyloidosis. (cdc.gov)
- Clarifying immunoglobulin gene usage in systemic and localized immunoglobulin light-chain amyloidosis by mass spectrometry. (cdc.gov)
- Genomic profiling in amyloid light-chain amyloidosis reveals mutation profiles associated with overall survival. (cdc.gov)
- Left ventricular assessment in patients with systemic light chain amyloidosis: a 3-dimensional speckle tracking transthoracic echocardiographic study. (cdc.gov)
- The syndrome also can be associated with a lymphoplasmacytic clone with an immunoglobulin M (IgM) gammopathy or with several other types of B monoclonal proliferation, including monoclonal gammopathy of unknown significance (MGUS). (cancernetwork.com)
- It is the monoclonal light chain immunoglobulin that is of particular importance in POEMS-in the vast majority of cases, a lambda light chain derived from two variable (V)-region germline genes, IGLV1-44*01 and IGLV1-40*01 . (cancernetwork.com)
- Conduct serum protein electrophoresis, immunofixation analyses, and kappa and lambda free light chain (FLC) assays in serum, to determine the age-adjusted prevalence and monoclonal protein size distribution of MGUS by ethnic/racial group. (cdc.gov)
- Malignant plasma cells in bone marrow produce an immunoglobulin, usually monoclonal IgG or IgA or, less commonly, immunoglobulin light chains . (who.int)
- Variations in the heavy polypeptide chain structure of gamma myeloma immunoglobulins from an inbred strain of mice and a hypothesis as to their origin. (wikidata.org)
- The biopsy sample of patient 10, who was reported to have multiple myeloma, was positive for light chains and β2 microglobulin. (opendentistryjournal.com)
- Two Ig light chains and two Ig heavy chains (IMMUNOGLOBULIN HEAVY CHAINS) make one immunoglobulin molecule. (bvsalud.org)
- It also reacts with the light chains of other rat immunoglobulins. (jacksonimmuno.com)
- By immunoelectrophoresis and ELISA this antibody reacts specifically with rabbit IgG and with light chains common to other rabbit immunoglobulins. (abcam.com)
- This goat polyclonal antibody reacts with the heavy (H) chains on mouse IgG and the light (L) chains common in most mouse immunoglobulins. (stemcell.com)
- No antibody was detected against non-immunoglobulin serum proteins. (jacksonimmuno.com)
- The antibody has been tested by ELISA and/or solid-phase adsorbed to ensure minimal cross-reaction with bovine, chicken, goat, guinea pig, syrian hamster, horse, human, rabbit and sheep serum proteins, but it may cross-react with immunoglobulins from other species. (jacksonimmuno.com)
- The normal reference range of 0.26 to 1.65 for the free-kappa-lambda ratio in the FLC assay reflects a higher serum level of free-lambda light chains than would be expected given the usual kappa-lambda ratio of two intact immuno¬globulins. (cdc.gov)
- serum immunoglobulin characteristics classify leukaemia into different levels (Potapnev et al . (who.int)
- Bence-Jones proteins are a part of regular antibodies called light chains. (medlineplus.gov)
- The basic structure of immunoglobulin (Ig) molecules is a tetramer of two light chains and two heavy chains linked by disulphide bonds. (embl.de)
- This entry represents a subtype of the immunoglobulin domain, and is found in a diverse range of protein families that includes glycoproteins, fibroblast growth factor receptors, vascular endothelial growth factor receptors, interleukin-6 receptor, and neural cell adhesion molecules. (embl.de)
- There are five types of heavy chains: alpha, delta, epsilon, gamma and mu, all consisting of a variable domain (VH) and three (in alpha, delta and gamma) or four (in epsilon and mu) constant domains (CH1 to CH4). (embl.de)
- This study therefore, evaluated the plasma levels of nitric oxide (NO), interleukin-4 (IL-4), interferon-gamma (IFN-) and immunoglobulin classes (IgA, IgG, IgM, IgE) in twenty-five (25) patients with acute leukaemia (AL) and twenty-five (25) apparently healthy controls. (who.int)
- Malignant plasma cells in meningeal MM out of 2000 patients with bone marrow produce an immunoglobulin, MM, was reported was by Schluterman et al. (who.int)
Kappa and lambda2
- Polypeptide chains, consisting of 211 to 217 amino acid residues and having a molecular weight of approximately 22 kDa. (bvsalud.org)
- Sometimes, when your body makes too many antibodies, the level of light chains also rises. (medlineplus.gov)
- It consists of two separate measurements, one to detect free-kappa (normal range, 3.3-19.4 mg/L) and the other to detect free-lambda (normal range, 5.7-26.3 mg/L) light chains. (cdc.gov)
- In addition to measuring the absolute levels of FLC, the test also allows the assessment of clonality based on the ratio of kappa-lambda light chain levels (normal reference range, 0.26-1.65). (cdc.gov)
- A single-chain variable fragment (scFv) is not actually a fragment of an antibody, but instead is a fusion protein of the variable regions of the heavy (VH) and light chains (VL) of immunoglobulins, connected with a short linker peptide of ten to about 25 amino acids. (graffletopia.com)
- In light of the recent outbreaks in the Americas, the number of Zika virus disease cases among travelers visiting or returning to the United States is likely to increase. (cdc.gov)
- Immunoglobulin-like domains that are related in both sequence and structure can be found in several diverse protein families. (embl.de)
- Congo red staining of a cardiac biopsy specimen containing amyloid, viewed under polarized light. (medscape.com)
- Immunoglobulins were detected with serially diluted Donkey Anti-Rabbit IgG(H+L)-FITC (SB Cat. (southernbiotech.com)
- It is a glycoprotein consisting of two identical heavy chains (50 kDa each) and two identical light chains (25 kDa each), to give a combined mass of approximately 150 kDa. (chxa.com)
- In the middle of each heavy chain is a relative variable portion called the "hinge region" that is unique to each IgG. (chxa.com)
- In the present study, we applied the oral swab technique in combination with quantification of organism-specific DNA using real-time polymerase chain reaction (PCR) to monitor the progression of infection in the rat model. (cdc.gov)
- 5. Evidence for the significant role of immunoglobulin light chains in antigen recognition and selection in chronic lymphocytic leukemia. (nih.gov)
- In the recognition phase of humoral immunity, the membrane-bound immunoglobulins serve as receptors which, upon binding of a specific antigen, trigger the clonal expansion and differentiation of B lymphocytes into immunoglobulins-secreting plasma cells. (nih.gov)
- The antigen binding site is formed by the variable domain of one heavy chain, together with that of its associated light chain. (nih.gov)
- Thus, each immunoglobulin has two antigen binding sites with remarkable affinity for a particular antigen. (nih.gov)
- They are defined for immunoglobulin allotypes, which are allelic variants of antibody heavy and light chains. (nih.gov)
- Surrogate light chains associate with MU IMMUNOGLOBULIN HEAVY CHAINS in place of a conventional immunoglobulin light chains to form pre-B cell receptors. (nih.gov)
- A discrepancy between the results of a urine dipstick test for protein and the findings from a test for 24-hour urine protein excretion should suggest the possibility of light-chain proteinuria. (medscape.com)
- Light-chain proteins in the urine cannot be detected using Albustix or other dipstick methods. (medscape.com)
- 11. Lack of intraclonal diversification in Ig heavy and light chain V region genes expressed by CD5+IgM+ chronic lymphocytic leukemia B cells: a multiple time point analysis. (nih.gov)
- The normal reference range of 0.26 to 1.65 for the free-kappa-lambda ratio in the FLC assay reflects a higher serum level of free-lambda light chains than would be expected given the usual kappa-lambda ratio of two intact immuno¬globulins. (cdc.gov)
- The antibody is a biochemically purified IgG 2a immunoglobulin with a heavy chain of approximately 50,000 daltons and a light chain of approximately 25,000 daltons. (nih.gov)
- A moderate degree of liver dysfunction may be observed because of the deposition of light chains in the liver or other organs. (medscape.com)
- An unusual case of light chain proximal tubulopathy (LCPT) without any usual sign of tubular cell dysfunction apart from mild proteinuria, but with a complete abolishment of tubular secretion of creatinine has been reported. (medscape.com)
- An immunolglobulin light chain-like protein composed of an IMMUNOGLOBULIN VARIABLE REGION-like peptide (such as light chain like lambda5 peptide) and an IMMUNOGLOBULIN CONSTANT REGION-like peptide (such as Vpreb1 peptide). (nih.gov)
- From UniProtKB: Constant region of immunoglobulin light chains. (nih.gov)
Heavy and light2
- The Putnam heat test can help detect urinary light chains only when the concentration exceeds 150 mg/dL. (medscape.com)
- 6. Similarities and differences between the light and heavy chain Ig variable region gene repertoires in chronic lymphocytic leukemia. (nih.gov)
- 8. Immunoglobulin light chain repertoire in chronic lymphocytic leukemia. (nih.gov)
- 9. Normal B cells express 51p1-encoded Ig heavy chains that are distinct from those expressed by chronic lymphocytic leukemia B cells. (nih.gov)
- If needed, plasma cells can be identified with immunohistochemistry for human kappa light chains or immunoglobulins. (nih.gov)
- The mutant ali/ali animals have a deletion in a key variable region gene in the immunoglobulin heavy chain locus that is present in the related wild type 2R1/2R1. (nih.gov)
- If a patient has a negative result from the Albustix test (which detects albumin) and a positive result from the SSA test, consider the possibility of light-chain proteinuria. (medscape.com)
- Hypotheses include frequent zoonotic infections with limited subsequent human-to-human transmission chains and existence of a self-sustained epidemic in humans ( 4 ). (cdc.gov)
- Secreted immunoglobulins mediate the effector phase of humoral immunity, which results in the elimination of bound antigens (PubMed:22158414, PubMed:20176268). (nih.gov)
- 2013 . Polyclonal free light chains: a biomarker of inflammatory disease or treatment target? (nih.gov)