Amyloidosis Samples

Bay Biosciences provides high quality, clinical grade, fresh frozen tumor tissue bio-specimens, cryogenically preserved sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluid samples from patients diagnosed with Amyloidosis.

The sera (serum), plasma and PBMC biofluid specimens are processed from patient’s peripheral whole-blood using customized collection and processing protocols. The amyloidosis fresh frozen tumor tissue samples and biofluids are collected from unique patients diagnosed with amyloidosis and are provided to a valued pharmaceutical customer for research, diagnostics, discovery and drug development.

Detailed clinical data, amyloidosis patients history, symptoms, complete blood count (CBC), serology, chemotherapy information, fresh frozen tumor tissue, elevated biomarker levels, genetic and metabolic information, histopathological findings, annotations associated with amyloidosis specimens is provided to a valued customer for research, development and drug discovery.

The amyloidosis sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluid are processed from patients peripheral whole-blood using customized collection and processing protocols.

Amyloidosis Overview

Amyloidosis is a rare and serious protein deposition disease. It is caused by an abnormal protein called amyloid that builds up in tissues or organs. As the amount of amyloid protein deposits increase in a tissue or organ, they interfere with the tissue or organ’s healthy function. Eventually, the amyloid protein deposits cause symptoms and organ failure, amyloidosis can be fatal.

Amyloid protein deposits, or amyloidosis, may be localized to organs, such as the lung, skin, bladder, or bowel, or they can be systemic which means that the deposits may be found throughout the body. Systemic amyloidosis is the most common. Although amyloidosis is not a type of cancer, it may be associated with certain blood cancers like multiple myeloma.

Types of Amyloidosis

Following are different types of amyloidosis disease:

Light chain (AL) amyloidosis: This is the most common type of amyloidosis disorder in the United States. The amyloid proteins that build up in the tissues in this condition are known as light chains. They can either be kappa or lambda light chains. AL amyloidosis is a disorder of the plasma cells. Plasma cells are a type of white blood cell responsible for the production of immunoglobulins or antibodies, which are a type of protein that fights infection. In AL amyloidosis, the light chain proteins are misshapen and produced in excess. They deposit in tissues and can damage one or more organs. The heart, kidneys, nerves, and gastrointestinal system are the most common organs affected. Because AL amyloidosis is associated with the overproduction of plasma cell proteins, it is linked to multiple myeloma.
Autoimmune (AA) amyloidosis: AA amyloidosis is also called secondary amyloidosis or inflammatory amyloidosis. In this condition, the amyloid protein that builds up in the tissues is called the A protein. AA amyloidosis is associated with some chronic diseases, such as diabetes, tuberculosis, rheumatoid arthritis, and inflammatory bowel disease. It may also be linked to aging. AA amyloidosis can affect the spleen, liver, kidneys, adrenal glands, and lymph nodes. Lymph nodes are tiny, bean-shaped organs that fight infection.
Hereditary or familial amyloidosis: Hereditary amyloidosis is rare inherited disorder, it can be passed from generation to generation within a family. The proteins produced in hereditary amyloidosis may cause problems with the heart and may cause carpal tunnel syndrome and eye abnormalities. The most common subtypes involve a protein called transthyretin (TTR).

This section covers AL, AA, and hereditary amyloidosis. Other types of amyloidosis include beta-2 microglobulin amyloidosis, which occurs in some patients with chronic kidney problems, and types of amyloidosis located in specific areas of the body.

Signs and Symptoms of Amyloidosis

The symptoms of amyloidosis can vary widely, depending on the specific organ or number of organs affected by the buildup of amyloid protein(s). Patients with amyloidosis may experience the symptoms or signs listed below. Sometimes, patients with amyloidosis do not have any of these changes. Or, the cause of a symptom may be another medical condition. All of these factors can make diagnosing amyloidosis challenging because the symptoms may be similar to those of numerous other diseases and conditions that are more common than amyloidosis.

Symptoms of amyloidosis are usually determined by the organ or function that is affected by the protein buildup. For example:

Kidneys: Amyloidosis in the kidneys will reduce the kidneys’ ability to filter waste and break down proteins. As a result, large amounts of protein may be found in the urine, causing foamy urine. The kidneys may even stop working. Decreased urine output and changes in creatinine clearance tests, a blood test to measure kidney function, may be present.
Liver: Amyloidosis may cause the liver to grow larger and affect its ability to function normally. This may cause pain in the upper abdomen, swelling in the abdomen, and changes in liver enzymes that can be found using blood tests.
Heart: Amyloidosis of the heart may cause an irregular heartbeat, called an arrhythmias, which enlarges the heart and cause poor heart function, resulting in fluid buildup, an irregular heartbeat, shortness of breath, or chest pain.
Gastrointestinal tract: Amyloidosis of the gastrointestinal tract may cause problems with the digestion and absorption of food nutrients, diarrhea or constipation, bleeding, blockages, and a thickened tongue, called macroglossia. It may also cause problems with the esophagus, including gastroesophageal reflux disease (GERD).
Thyroid gland: Amyloidosis of the thyroid gland may cause goiter, a noncancerous swelling of the thyroid gland.
Lungs: Amyloidosis of the lungs may cause problems with breathing, including shortness of breath.
Nervous system: Disorders of peripheral nerves are the most common neurological complications of amyloidosis. Patients may experience painful paresthesias (unusual sensations), numbness and balance difficulties, vomiting, diarrhea, constipation, sweating, or sexual problems. Numbness, tingling, or weakness in the arms or legs may develop. This condition is known as peripheral neuropathy. Carpal tunnel syndrome may also occur.

Other general symptoms of amyloidosis include:

Fatigue: Fatigue is extreme exhaustion or tiredness, it is a common problem for people with amyloidosis. Patients who feel fatigue often say that even a small effort, such as walking across a room, can seem like too much.
Unexplained weight loss
Anemia (low level of red blood cells)
Weak hand grip, which may arise from carpal tunnel syndrome
Skin changes, such as a rash around the eyes
Clay-colored stools
Joint pain
Risk of developing infection: Patients with AL amyloidosis are at increased risk of getting an infection. This is because this type of amyloidosis causes abnormalities in plasma cells. Plasma cells produce immunoglobulins, which help fight infection. A condition called leukopenia occurs when the body does not have enough white blood cells and is less able to fight off infections. Some white blood cells, called neutrophils, can destroy harmful bacteria and fungi. Patients with leukopenia or neutropenia, a low level of neutrophils, are more likely to develop a serious infection.

Risk Factors of Amyloidosis

Most patients who develop amyloidosis have no known risk factors. The cause is often not known. The following factors may increase a person’s risk of developing amyloidosis:

Age: The risk of amyloidosis increases as a person gets older. For AL amyloidosis, the majority of patients diagnosed are older than 40 years old.
Gender: Amyloidosis is more common in men than in women.
Other diseases: Amyloidosis is sometimes linked with another disease, for example, research shows that 12% to 15% of people with multiple myeloma also develop AL amyloidosis.
Family history: Hereditary amyloidosis can run in families. This may be due to a genetic change, called a mutation, that is passed down from generation to generation.

Diagnosis of Amyloidosis

In addition to a physical examination, the tests listed below may be used to diagnose amyloidosis. After these diagnostic tests are done, your doctor will review all of the results with you to decide on the treatment.

Laboratory tests: Doctors may take samples of the patient’s blood and urine to run tests to learn more about the patient’s disease and general health.
Bone marrow aspiration and biopsy: These two procedures are similar and often done at the same time to examine the bone marrow. A common site for bone marrow biopsy and aspiration is the posterior iliac crest, a part of the pelvic bone located in the lower back. Bone marrow is the spongy tissue inside your bones. It has both a solid and a liquid part. A bone marrow aspiration removes a sample of the fluid with a needle. A bone marrow biopsy is the removal of a small amount of solid tissue using a needle. A pathologist then analyzes the sample(s).
Biopsy: A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that amyloid proteins are present, but only a biopsy can make a definite diagnosis. A pathologist then analyzes the sample(s). A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease. When amyloidosis is suspected, tissue samples are most often taken from abdominal fat or bone marrow. A sample may also be taken from the liver, nerves, heart, kidneys, or rectum. However, these are more invasive procedures, and a patient may need to stay in the hospital for these tests.
Ultrasound: An ultrasound uses sound waves to create a picture of the internal organs. Areas affected by amyloid protein make different echoes of the sound waves than healthy tissue. When the sound waves bounce back to the computer and are changed into images, the doctor can find these areas inside the body. An ultrasound of the abdominal area may be used to look for enlarged organs.
Heart evaluation: A heart evaluation, including an electrocardiogram (EKG or ECG) and an echocardiogram (echo), will look for structural abnormalities in the heart and examine the motion of the walls of the heart.

Treatment of Amyloidosis

Different types of doctors often work together to create a patient’s overall treatment plan that combines different types of treatments. This is especially important because amyloidosis affects so many different organs.

The amyloidosis treatment team often includes the following experts:

Hematologist: A doctor who specializes in blood disorders
Cardiologist: A doctor who treats heart conditions
Gastroenterologist: A doctor who specializes in conditions of the gastrointestinal tract
Pulmonologist: A doctor who specializes in conditions of the lungs
Nephrologist: A doctor who treats kidney problems
Neurologist: A doctor who focuses on issues involving the brain and nervous system

Following are the most common treatment options for amyloidosis:

Medications to Treat Amyloidosis

Medication may be used to control the problems associated with amyloidosis. Steroids have been shown to work well, and they are typically combined with other drugs.

Chemotherapy

Chemotherapy is the use of drugs to destroy abnormal cells, usually by stopping the cells’ ability to grow and divide. It is most commonly used to treat cancer. However, chemotherapy is also useful for other conditions, including amyloidosis. Chemotherapy may be given by a hematologist or a medical oncologist, a doctor who specializes in giving chemotherapy to treat cancer.

During treatment for amyloidosis, chemotherapy is used to destroy abnormal cells in the blood. Common ways to give chemotherapy include an intravenous (IV) tube placed into a vein using a needle, a shot under the skin, or a pill or capsule that is swallowed (orally). A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a set period of time. A patient may receive one drug at a time or combinations of different drugs at the same time.

Some of the common types of chemotherapy used to treat amyloidosis are cyclophosphamide (Cytoxan, Neosar) and melphalan (Alkeran) combined with dexamethasone (multiple brand names) and prednisone.

Side effects of chemotherapy

The side effects of chemotherapy depend on the individual and the dose used, but they can include nausea, vomiting, and fatigue. These are generally manageable when they do occur, and they usually go away once treatment is finished. Some types of chemotherapy may cause uncommon long-term side effects, including damage to the bone marrow and other organs.

Anemia is common in patients with amyloidosis, especially those who are receiving chemotherapy, such as melphalan. Anemia is an abnormally low level of red blood cells (RBCs). RBCs have an iron-containing protein called hemoglobin that carries oxygen to all parts of the body. If the level of RBCs is too low, parts of the body do not get enough oxygen and cannot work properly. Most people with anemia feel tired or weak.

Targeted Therapy

Targeted therapy is a treatment that targets specific genes, proteins, or the tissue environment that contributes to the development of amyloidosis. This type of treatment blocks the growth and spread of abnormal cells while limiting damage to healthy cells.

For amyloidosis, targeted therapies include anti-angiogenesis therapy, monoclonal antibodies, and proteasome inhibitors.

Immunomodulatory drugs: Drugs in this class include lenalidomide (Revlimid), pomalidomide (Pomalyst), and thalidomide (Synovir, Thalomid).
Monoclonal antibodies: A monoclonal antibody is a type of targeted therapy. It recognizes and attaches to a specific protein in the abnormal cells, and it does not affect cells that don’t have that protein. Drugs in this class include daratumumab (Darzalex) and elotuzumab (Empliciti). A second type of monoclonal antibody directly targets the amyloid itself. There are currently several of these antibodies in clinical trials.
Proteasome inhibitors: Proteasome inhibition is a type of targeted therapy. The drugs in this class target specific enzyme called proteasomes that digest proteins in the cells. This class includes bortezomib (Velcade), ixazomib (Ninlaro), and carfilzomib (Kyprolis).

Researchers continue to study these drugs to find out how well each treats amyloidosis. These drugs do not reverse the buildup of amyloid proteins in tissues or organs. However, studies researching the combination of bortezomib, dexamethasone, and melphalan have shown that they may help the organs affected by amyloidosis work better. Recent studies have started to examine the role of treatments that can improve organ function by directly targeting the amyloid deposits. This type of treatment is still being studied in clinical trials.

Side effects of targeted therapy

Targeted therapy may cause different side effects from those usually linked with chemotherapy. Patients receiving targeted therapy may develop skin, hair, nail, and/or eye problems.

Surgical Procedure

Surgery used to treat amyloidosis may include organ transplantation. Liver transplantation has been effective in treating certain types of hereditary amyloidosis. Kidney and heart transplantation may also work well. However, organ transplantation may not be appropriate for all patients, especially those who have large amounts of amyloid proteins that have been deposited in their organs.

Stem cell transplantation/bone marrow transplantation

A bone marrow/stem cell transplant is a medical procedure in which the plasma cells in the bone marrow that produce amyloid protein are first destroyed by high doses of chemotherapy and then replaced by highly specialized cells, called hematopoietic stem cells, that develop into healthy bone marrow. Hematopoietic stem cells are blood-forming cells found both in the bloodstream and in the bone marrow. Today, this procedure is more commonly called a peripheral blood stem cell transplant, rather than bone marrow transplant, because it is the stem cells collected from the bloodstream that are typically being transplanted, not the actual bone marrow tissue.

For some patients with amyloidosis who have some normal organ function, peripheral blood stem cell transplantation may be useful. During this procedure, the patient is given high doses of chemotherapy to destroy blood cells that are making the amyloid proteins. The patient then receives healthy peripheral blood stem cells, which allow the body to start making healthy blood cells again. Survival can be significantly improved with high-dose chemotherapy and peripheral blood stem cell transplantation. However, many patients cannot receive this treatment because the amyloid protein buildup has affected the function of other organs.

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