Medical Terminology

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Albumin

A protein which constitutes more than half of the blood's serum. Where amyloid has affected the kidney's filtration system albumin may pass into the urine.

Alkylating Agent

A chemotherapeutic agent such as melphalan or cyclophosphamide. Alkylating refers to the way in which these agents cross-link the DNA of myeloma cells and block cell division.

Allogeneic stem cell transplant

A procedure in which stem cells or bone marrow from a compatible tissue type donor (usually a family member) are collected, stored and given to the patient after high dose chemotherapy treatment. Usually donors are HLA identical family members. HLA refers to the Histocompatiability Locus Antigens used for tissue matching. (Unrelated Allogeneic Transplant - the person donating is unrelated to the patient).

Amyloidosis

A condition in which myeloma light chains (Bence Jones proteins) are deposited in tissues and organs throughout the body. This occurs more commonly with lambda versus kappa Bence Jones proteins. In patients with amyloidosis, the light chain proteins bind to certain tissues such as heart, nerves and kidney rather than being excreted out of the body through the kidneys.

Anaemia

A below-normal number of red blood cells in the blood. This reduces the ability of the blood to supply oxygen to the body, causing fatigue and weakness.

Angiogenesis

Blood vessel formation, which usually accompanies the growth of malignant tissue, including myeloma.

Antibiotics

Drugs used to treat bacterial infections.

Antibody

A protein produced by certain white blood cells (plasma cells) to fight infection and disease in the form of antigens such as bacteria, viruses, toxins, or tumours. Each antibody can bind only to a specific antigen. The purpose of this binding is to help destroy the antigen. Antibodies can work in several ways, depending on the nature of the antigen. Some antibodies disable antigens directly. Others make the antigen more vulnerable to destruction by other white blood cells.

Anti-emetics

Drugs to prevent or minimize nausea and vomiting.

Antigenic

Having the properties of an antigen. Antigens are a vital component of the immune system.

Antigens

Any substance that the body regards as foreign or potentially dangerous, which can be as diverse as bacteria and pollen grains, against which it produces an antibody.

Apheresis

A procedure in which stem cells are collected from the blood using a machine which separates them out, returning the remainder of the blood components to the donor. As stem cells are within the white blood cell components it is also sometimes referred to as leukapheresis.

Apoptosis

A normal cellular process involving a genetically programmed series of events leading to the death of a cell.

Arsenic trioxide

A potential new treatment for myeloma undergoing clinical studies.

Aspiration

The process of removing fluid or tissue, or both, from a specific area.

Asymptomatic

Without symptoms.

Autologous stem cell transplant

A procedure in which a patient's own stem cells are collected, stored and then given back following high dose chemotherapy. This is the most common form of transplantation used in myeloma.

 

 

Bence Jones Protein

A myeloma protein present in urine, used to identify immunoglobulin or antibody fragments kappa or lambda light chains) present in the urine. The amount of Bence Jones protein is expressed in terms of grams per 24 hours. Normally averysmallamountofprotein(<0.1g/24h) can be present in the urine, but this is albumin rather than Bence Jones protein. The presence of any Bence Jones protein is abnormal.

Beta 2 Microglobulin (ß2M)

A small protein found in the blood. High levels occur in patients with active myeloma. Low or normal levels occur in patients with early myeloma and / or inactive disease. Approximately 10% of patients have myeloma that does not produce ß2M. For these patients, ß2M testing cannot be used to monitor the disease. At the time of relapse, ß2M can increase before there is any change in the myeloma protein level. Therefore, 90% of the time, ß2M is very useful for determining disease activity.

Bias

Human choices or any other factors beside the treatments being tested that affect a study’s results. Clinical studies use many methods to avoid bias, because biased results may not be correct.

Biopsy

The removal of a small sample of living tissue for microscopic examination to aid in diagnosis.

Bisphosphonates

A type of drug that binds to the surface of bone and protects against bone breakdown by bone-eating cells.They include clodronate (Bonefos), pamidronate (Aredia) and zoledronic acid (Zometa). In myeloma, they are used to treat bone disease and a high level of calcium in the blood (hypercalcaemia).

Blood cells

Minute structures produced in the bone marrow; they consist of red blood cells, white blood cells, and platelets.

Blood count

The number of red blood cells, white blood cells, and platelets in a sample of blood.

Bone marrow

The soft, spongy tissue in the centre of bones that produces white blood cells, red blood cells, and platelets.

Bone marrow aspiration

The removal, by a needle, of a sample of fluid and cells from the bone marrow for examination under a microscope.

Bone marrow biopsy

The removal, by a needle, of a sample of tissue from the bone. The cells are checked to see whether they are cancerous. If cancerous plasma cells are found, the pathologist estimates how much of the bone marrow is affected. Bone marrow biopsy is usually done at the same time as bone marrow aspiration.

Bortezomib (Velcade)

Proteasome inhibitor treatment for myeloma, undergoing clinical studies in the UK.

 

 

Carpal Tunnel Syndrome

A condition where there is a compression of the median nerve in the wrist as the nerve passes through the carpal tunnel. Causing pain, numbness, or tingling in the fingers.

CAT (Computerised Axial Tomography) scan

A test using Computerised X-rays to create three-dimensional images of organs and structures inside the body, used to detect small areas of bone damage or soft tissue involvement. Also called CT (Computed Tomography) scan.

Catheter

A tube that is placed in a blood vessel to provide a pathway for drugs or nutrients. A Central Venous Catheter is a special tubing that is surgically inserted into a large vein near the heart and exits from the chest or abdomen. The catheter allows medications, fluids, or blood products to be given and blood samples to be taken.

Cell proliferation

An increase in the number of cells as a result of cell growth and cell division.

Chemotherapy

Treatment with potent/cytotoxic drugs intended to kill cancer cells. Chemotherapy can be injected into a vein (intravenous or IV) or swallowed as tablets (orally).

Chromosome

A strand of DNA and proteins in the nucleus of a cell. Chromosomes carry genes and function in the transmission of genetic information. Normally, human cells contain 46 chromosomes.

Clinical

Refers to the treatment of humans, as opposed to animals or laboratory studies.

Clinical study / trial

A research study of new treatment that involves patients following favourable results in laboratory experiments. Each study is designed to find better ways to prevent, detect, diagnose, or treat cancer and to answer scientific questions.

Congestive Heart Failure

A condition where ineffective pumping of the heart leads to an accumulation of fluid in the lungs.

Congo Red

A dye used for the detection of Amyloidosis through its ability to stain the abnormal amyloid deposits.

Control group

In a clinical study, the group of people that receives standard treatment for their cancer.

Conventional / standard treatment

The best treatment currently used based on results of past research.

Creatinine

A small chemical compound normally excreted by the kidneys. If the kidneys are damaged, the serum level of creatinine builds up, resulting in an elevated serum creatinine. The serum creatinine test is used to measure kidney function.

Creatinine Clearance

A sensitive test of kidney function that requires a 24-hour urine sample and a blood sample. The test is often required to make sure it is safe to give anticancer drugs that may be toxic to the kidneys.

CT or CAT scan

(Computed Tomography scan or Computed Axial Tomography scan). Detailed pictures of areas of the body created by a computer linked to an x-ray machine.

Cytokine

A substance secreted by cells of the immune system that stimulates growth / activity in a particular type of cell. Cytokines are produced locally (i.e. in the bone marrow) and circulate in the bloodstream.

Cytotoxic

Destroys cells.

 

 

DEXA Scan

(Dual Photon X-ray Absorptionmetry) study

Measures the amount of bone loss; the best measure of bone density.

Dexamethasone

A powerful steroid given alone or with other chemotherapy drugs in the treatment of blood disorders.

Dialysis

When a patient’s kidneys are unable to filter blood, the blood is cleaned by passing it through a dialysis machine.

Diuretic

An agent that promotes the excretion of urine. Often used in the treatment of oedema and high blood pressure.

DMSO

Dimethyl sulfoxide, a colourless chemical used in the storage of stem cells.

DNA

(Deoxyribonucleic acid) The hereditary material in humans and almost all other organisms

Double blind study

Is when neither the doctor or the patient knows who is in which study group.

 

 

Echo Cardiogram

A test which uses high frequency sound waves to image the heart and surrounding tissues.

Electrocardiogram (ECG)

Recording of the electrical activity of the heart on a moving strip of paper.

Electrophoresis

A laboratory test in which a patient’s serum (blood) or urine molecules are subjected to separation according to their size and electrical charge. For myeloma patients, electrophoresis of the blood or urine allows both the calculation of the amount of myeloma protein (M-protein) as well as the identification of the specific M-spike characteristic for each patient. Electrophoresis is used as a tool both for diagnosis and for monitoring.

End points

An event used by a clinical study to evaluate whether or not a study therapy is working, e.g. in myeloma, achieving remission or a fall in paraprotein below a certain level.

Engraftment

The process by which the transplanted stem cells travel to the recipient's bone marrow, where they will begin to grow and develop into new blood cells. During this time the number of red cells, white cells and platelets in the blood may be lower than normal.

Enzyme

A substance that affects the rate at which chemical changes take place in the body.

Erythrocytes

Red blood cells (RBCs). RBCs carry oxygen in the form of haemoglobin to body cells and carbon dioxide away from body cells.

Erythropoeitin (EPO)

A hormone produced by the kidneys necessary for the production of red blood cells. Where a kidney is damaged injections with synthetic erythropoietin can be helpful. Blood transfusion is another alternative especially in an emergency. Synthetic erythropoietin is being used prophylactically before chemotherapy and as a supportive therapy after chemotherapy to avoid anaemia.

 

 

Free Light Chains

A portion of the monoclonal protein of light molecular weight that can be measured in a sensitive assay, the Freelite™ test.

 

 

Gamma Globulin

Proteins in the blood that contain antibodies, part of the body's defence against infection.

Gene

A unit of genetic material (DNA) that carries the directions a cell uses to perform a specific function, such as making a given protein.

Genetic

Inherited, having to do with information that is passed from parents to their children through DNA.

Graft-versus-host disease (GvHD)

A complication of allogeneic transplants whereby the donor cells (the graft) recognises the recipient's body (the host) as foreign and mounts an attack. This can cause skin, liver and gut problems, and is usually treated with steroids.

Graft-versus-myeloma

A beneficial effect of an allogeneic transplant which may result from the donor cells mounting an attack on the recipient's myeloma cells.

Growth Factor

A protein that stimulates the development and growth of cells. Granulocyte colony stimulating factor (G-CSF) is a growth factor used to stimulate the growth of stem cells before collection.

 

 

Haemoglobin

The molecule that carries oxygen around the body in red blood cells

Hepatic

Pertaining to the liver.

Hepatomegaly

Enlargement of the liver.

HICKMAN® /Groshong catheter/line

A catheter (tube) that is inserted into a large vein in the neck, and then tunnelled under the skin. Can be kept in for several months and is necessary in order to be able to give the chemotherapy and other medications required when having a stem cell transplant.

Hypercalcaemia

A higher-than-normal level of calcium in the blood. This condition can cause a number of symptoms, including loss of appetite, nausea, thirst, fatigue, muscle weakness, restlessness, and confusion. Common in myeloma patients and usually resulting from bone destruction with release of calcium into the blood stream. Often associated with reduced kidney function since calcium can be toxic to the kidneys. For this reason, hypercalcaemia is usually treated on an emergency basis using IV fluids combined with drugs to reduce bone destruction along with direct treatment for the myeloma.

 

 

IMiDs

A group of new drugs undergoing clinical studies, which are similar to thalidomide in action, but may have fewer side effects.

Immune Response

The reactions of the immune system to foreign substances.

Immune System

The complex group of cells and organs that defend the body against infection and disease.

Immunofixation

A technique used to identify specific proteins in the blood or urine. It has greatest application in the identification (and monitoring) of monoclonal proteins, such as those produced by AL Amyloidosis.

Immunoglobulins

Also known as antibodies, immunoglobulins are proteinsfound in the blood which are produced by cells of the immune systems, called plasma cells. Their function is to bind to substances in the body that are recognised as foreign antigens found on the surface of bacteria and viruses, most commonly to fight infection. There are five main types; IgA, IgD, IgE, IgG and IgM.

Immunosuppression

Suppression of the immune system, which can mean an increased risk of infection. Immunosuppressive drug Medications given to suppress the patient's immune system, and help prevent rejection of donor stem cells.

Induction chemotherapy

The initial chemotherapy treatment given to treat myeloma

Informed Consent

The process requiring a doctor to give a patient enough information about a proposed procedure for the patient to make an informed decision about whether or not to undergo it. The doctor must, in addition to explaining all procedures, address the issues of risks, benefits, alternatives, and potential costs.

Interferon

A naturally produced hormone (cytokine) released by the body in response to infection or disease which stimulates the growth of certain disease fighting blood cells in the immune system. Interferon can be artificially produced by genetic engineering techniques and used as a form of immunotherapy, primarily in the maintenance (plateau) phase to block any re-growth of myeloma and thus delay or prevent relapse.

Interferon alpha

Is a synthesised version of a substance that is made by some white blood cells as part of the immune response. It works specifically on myeloma cells by interfering with how the cells grow and multiply. It also stimulates the immune system to attack the myeloma cells.

Interventional Radiologist

Interventional Radiologists specialise in invasive procedures that can biopsy internal organs, open blocked arteries and veins, drain abscesses and cysts and treat many other conditions and / or disorders. Interventional Radiologists utilise the results of many of the radiologic procedures. In many interventional radiology procedures, patients are treated on an outpatient basis and are back to their normal routines quickly.

Intravenous

Into a vein, a way of injecting drugs.

Investigator

A researcher in a treatment study.

 

 

Kidney Failure

Malfunction of the kidneys due to disease or the toxic effects of drugs or chemicals. Urine volume may or may not be diminished.

 

 

Lenalidomide (RevlimidTM)

Chemically similar to thalidomide, lenalidomide (RevlimidTM) is an immunomodulatory drug (IMiD) that works by affecting and modifying the immune system. The exact way in which IMiDs work is not yet fully understood, but like thalidomide, it is thought they have multiple mechanisms of action.

Lesion

An area of abnormal tissue change. A lump or abscess that may be caused by injury or disease, such as cancer. In myeloma, "lesion" can refer to a plasmacytoma or a hole in the bone - lytic lesion.

Leukocytes

Cells that help the body fight infections and other diseases. Also called white blood cells (WBCs).

Leukopenia

A low number of white blood cells.

Light Chain

The lighter of two types of chains that are found in immunoglobulins. There are two types of light chain, kappa and lambda, Amyloidosis may be described as kappa light chain or lambda light chain Amyloidosis.

Lymphocytes

White blood cells that fight infection and disease.

Lytic lesions

The damaged area of a bone that shows up as a dark spot on an Xray when enough of the healthy bone in any one area is eaten away. Lytic lesions look like holes in the bone and are evidence that the bone is being weakened.

 

 

M Protein

Simply means monoclonal protein (or M component/ paraprotein).

M proteins (M spike)

The over production of immunoglobulin protein from a single clone of plasma cells (monoclonal).The abnormal monoclonal product is called a monoclonal or M protein or paraprotein, and can show in serum or urine. Monoclonal or "M spike" refers to the sharp or spiked pattern which occurs on protein electrophoresis when an M protein is present. In Myeloma and AL Amyloidosis a plasma cell clone gives rise to clonal immunoglobulins or parts of immunoglobulins (ie kappa or lambda light chains) which show as an M spike in the electrophoresis pattern.

Macroglossia

Enlargement of the tongue.

Maintenance treatment

The treatment given after chemotherapy or transplantation that aims to prolong the period of response

MGUS

Monoclonal Gammopathy of Uncertain Significance – MGUS – is a premalignant disorder characterised by the accumulation of plasma cells within the bone marrow and the presence of a monoclonal protein spike on electrophoresis. The feature that distinguishes it from myeloma is the lack of end organ damage. What this means is that there are no lytic bone lesions, no renal damage and no anaemia. The condition is stable but by 10 years of follow up approximately 20% of patients will have progressed to clinical myeloma.

Mini-allogeneic transplant

A type of allogeneic transplant that uses lower doses of chemotherapy than a standard allogeneic transplant, and avoids some of the side effects and risks associated with higher-dose chemotherapy.

Mobilisation

The process by which the amount of stem cells in the bone marrow are increased in number, so that they 'spill over' into the blood stream and can be collected and stored.

Monoclonal

A clone or duplicate of a single cell. Myeloma develops from a single malignant plasma cell (monoclone). The type of myeloma protein produced is also monoclonal; a single form rather than many forms (polyclonal). The important practical aspect of a monoclonal protein is that it shows up as a sharp spike (M spike) in the serum electrophoresis test.

MRI (Magnetic Resonance Imaging)

Fine resolution pictures of areas of the body created by use of magnetic energy rather than x-ray, useful for soft tissue images.

Mucositis

Inflammation of the lining of the digestive tract, often seen as soreness of the mouth.

Multiple Myeloma

A haematological malignancy in which a clone of plasma cells causes bone damage and pain, low blood cell counts, increased infections and kidney damage.

 

 

Neoplasm

A new growth of tissue or cells; a tumour that can be referred to as benign or malignant.

Nephrotic Syndrome

An abnormal condition of the kidney that is characterised by low serum albumin, a large amount of protein in the urine and swelling (oedema).

Neutropenia

A reduced level of neutrophils or white blood cells.There are several types of white blood cells, and neutropenia refers to a reduction in the granulocytes, or neutrophils, necessary to adequately combat bacterial infections. Cytoxic chemotherapy has a tendency to induce neutropenia. In contrast lymphocytes which are more important in virus infections, tend not to be affected by cytotoxic treatment. Neutropenia can be prevented or reduced using a synthetic hormone called G-CSF (e.g. Neupogen®).

Neutrophils

A type of white blood cell necessary to combat bacterial infection.

 

 

Oedema

The presence of abnormally large amounts of fluid in the intercellular tissue spaces of the body, causing swelling.

Oncologist

A doctor who specialises in treating cancer.

Osteoblast

Bone forming cells.

Osteoclast

A cell found in the bone marrow at the junction between the bone marrow and the bone that resorbs or breaks down old bone. In myeloma, the osteoclasts are over-stimulated while osteoblast activity is blocked. The combination of accelerated bone resorption and blocked new bone formation results in lytic lesions.

Osteonecrosis of the jaws

A condition in which the bones of the jaws do not heal properly, causing ongoing, sometimes painful, complications.

 

 

Paraprotein

An antibody-like protein produced by the abnormal plasma cell in myeloma. It is found in the blood and sometimes also in the urine. It is also called monoclonal protein, myeloma protein, M spike, M protein or M band.

Peripheral Blood Stem Cells (PBSC)

Stem cells are collected from the circulating blood system, not the bone marrow.

Peripheral neuropathy

Damage to the peripheral nerves, particularly in the hands and feet, causing pain, tingling and altered sensation

Placebo

A tablet, capsule, or injection that looks like the drug or other substance being tested but contains no drug.

Plasma

The liquid part of the blood in which red blood cells, white blood cells, and platelets are suspended.

Plasma Cell

Produced by B lymphocyte (white blood cell) as a response to infection. Plasma cells are found in the bone marrow, connective tissue, and sometimes blood. Normal plasma cells produce antibodies to fight infection. In myeloma, the malignant plasma cells produce large amounts of a single abnormal antibody that does not fight infection. Malignant plasma cells also produce other chemicals that can result in organ and tissue damage.In AL Amyloidosis abnormal plasma cells produce abnormal antibodies which go onto form amyloid. The abnormal antibodies are the monoclonal protein, or M protein.

Plasmacytoma

A collection of myeloma plasma cells found in a single location rather than diffusely throughout the bone marrow, soft tissue, or bone.

Plasmapheresis

The process of removing certain proteins from the blood. Plasmapheresis can be used to remove excess antibodies from the blood of multiple myeloma patients.

Platelet

One of three major blood cells, others being red and white cells. Platelets plug up breaks in the blood vessel walls and stimulate blood clot formation. Platelets are the major defence against bleeding.

Progression-Free Survival

The improved survival of a patient that can be directly attributed to the treatment given for the AL Amyloidosis. The term identifies amyloid patients who are in complete remission versus those who have had an episode of relapse (or progression).

Protocol

An action plan for a clinical study. The plan states what will be done in the study and why. It outlines how many people will take part, what tests they will receive and how often, and the treatment plan.

 

 

Radiation therapy (Radiotherapy)

Treatment with X-rays, gamma rays, or electrons to damage or kill malignant cells. The radiation may come from outside the body (external radiation) or from radioactive materials placed directly in the tumour (implant radiation).

Randomisation

A method used to prevent bias in research. People are assigned by chance to either the treatment or control group.

Red Cells

One of three major types of cells in the blood. The blood cell which contains haemoglobin and carries oxygen from the lungs to all parts of the body. A low level of red cells is called anaemia. Red cell production is stimulated by a hormone called erythropoietin. Erythropoietin is produced by the kidneys.

Refractory disease

Refractory disease is disease that has failed to respond to previous treatments

Relapse

Relapsing disease is disease that has responded to a prior treatment but is now showing signs of returning

Remission

When the signs and symptoms of cancer go away, the disease is said to be ‘in remission’. A remission can be temporary or permanent.

Renal

Pertaining to the kidneys.

Response or Remission

Complete Remission or Complete Response (CR): Remission and response are usedinterchangeably. CR is the common abbreviation for both. CR is the absence of amyloid protein from the serum and/or urine by standard testing.

Partial Remission or Partial Response (PR): PR is a level of response less than CR.

 

 

SAP Scan

A nuclear medicine scanning procedure that can be used to ascertain the full extent of amyloid deposition in the body.

Side effects

Problems that occur when treatment affects healthy cells. Common side effects of standard cancer treatments are fatigue, nausea, vomiting, decreased blood cell counts, hair loss and mouth sores. New treatments being tested may have these or other unknown side effects.

Single blind study

A method used to prevent bias in treatment studies. In a single blind study, the patient is not told whether he / she is taking the standard treatment or the new treatment being tested. Only the doctors know.

Smouldering myeloma

In this condition there is minimal end organ damage but the level of plasma cells in the bone marrow and the paraprotein level are not stable and are associated with inexorable progression to myeloma. Conventionally, treatment is withheld until there is significant evidence of disease progression.

Spleen

An organ in the abdomen that plays an important role in immune system activities. It is part of the lymphatic system.

Stage

The extent of the cancer and whether the disease has spread from the original site to other parts of the body. Numbers with or without letters are used to define cancer stages (e.g. stage IIb).

Standard chemotherapy

Conventional dose chemotherapy treatment, given alone or in combination with other chemotherapy drugs and/or steroids.

Standard treatment

The best treatment currently known for a cancer, based on results of past research.

Stem cells

The immature cells from which all blood cells develop. Normal stem cells give rise to normal blood components, including red cells, white cells, and platelets. Stem cells are normally located in the bone marrow and can be harvested for transplant.

Study group

In a clinical study, the group of people that receives the new treatment for their cancer.

Syngeneic transplant

An allogeneic transplant which uses a patient's identical twin as their donor.

Syngenic

An identical twin donates the bone marrow or stem cells.

 

 

Tandem transplant

A planned double transplant procedure. It can be two autologous stem cell transplants, or an autologous followed by a mini allogeneic transplant.

Thalidomide

A new treatment that has been found to be effective in treating myeloma. It now being used and studied at all stages of myeloma. This drug was originally withdrawn in the 1960s because of birth defects caused when it was used as a treatment for morning sickness in pregnancy. Its prescription is now subject to a strict risk management programme.

Thrombocytopenia

A low number of platelets in the blood. The normal level is 150,000- 250,000. If the platelet count is less than 50,000, bleeding problems could occur. Major bleeding is usually associated with a reduction to less than 10,000.

Transplantation

In myeloma, stem cell transplants are done to allow the bone marrow to recover after high-dose chemotherapy treatment is given. Stem cells are taken from blood or bone marrow, and, following high-dose chemotherapy treatment, these stem cells are given back to renew the patient's blood-forming cells. Transplant is not a treatment, but a method of support to make high dose treatment possible.

Treatment group

The group that receives the new treatment being tested during a study

 

 

Vaccine

A drug administered for the prevention or treatment of infectious diseases.

 

 

Waldenström's macroglobulinaemia

A rare type of indolent lymphoma that affects plasma cells. Excessive amounts of IgM protein are produced. Not a type of myeloma.

White Blood Cell

One of three major types of cells in the blood. There are several types of white cells (i.e. neutrophils, lymphocytes and monocytes). Neutrophils are necessary to combat bacterial infection. Neutrophils can drop to very low levels following chemotherapy causing neutropenia. Neutropenia can be prevented or reduced using a synthetic hormone called G-CSF.

 

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