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Titlesort ascending Definition
x-linked genes Genes which are present on the X chromosome.
Wiskott-Aldrich Syndrome An X-linked primary immune deficiency that results in a low platelet count (thrombocytopenia), a recurring skin irritation called eczema, and increased susceptibility to infection. Because it is an x-linked disorder, this genetic condition is most often found in males. Its symptoms resemble those of leukemia; diagnosis may be confirmed through blood tests or genetic analysis. The genetic defect results in a protein mutation and subsequent immunodeficiencies. Treatment with IVIG boosts the immune system of WAS patients. Other treatment components may include platelet transfusions, splenectomy, and, in severe cases, bone marrow transplantation.
von Willebrand's Disease (vWD) A congenital bleeding disorder in women which passes genetically as an autosomal recessive trait. It is caused by deficient or defective von Willebrand factor (Factor III), a blood protein that controls platelet activity. The bleeding tendency manifests at an early age. Easy bruising, bleeding in the intestinal tract during surgery, and excess loss of blood during menstruation are common. It is the most common form of bleeding disorder. It is named for the Finnish physician Erik von Willebrand who discovered it. It is also called angiohemophilia and vascular hemophilia.
Varicella Zoster Virus (Vzv) A virus in the herpes family that causes chicken pox during childhood and may reactivate later in life to cause shingles in immunosurpressed individuals.
Variant Creutzfeldt-Jakob Disease A variant of Creutzfeldt-Jakob Disease (CJD). Both are diseases that affect the central nervous system. vCJD is a spongiform encephalopathy, meaning that as the disease progresses, it forms holes in the brain. It results in rapid and progressive loss of muscle control, dementia, memory loss, hallucinations, speech impairments, and difficulty walking and talking. There is no cure for vCJD, and it is always fatal, usually within a year of onset. vCJD is believed to be caused by a type or prion, a protienaceous infectious particle that is responsible for several diseases of the central nervous system. Variant Creutzfeldt-Jakob Disease (vCJD) is caused by a prion similar to that of CJD, and it has related symptoms, but affects a much younger population, with an average age of onset of 50 years of age. Bovine Spongiform Encephalopathy, or mad cow disease, is thought to be caused by a prion similar to that of vCJD and CJD. Causes of vCJD have been linked to contaminated cow meat, but no connection has been found between mad cow disease and CJD.
Vapor Treated A purification process useful for the sterilization of plasma-derived products such as blood coagulation factors and intravenous immune globulins. The plasma is treated with vapor to kill viruses.
TPN See: Total Parenteral Nutrition
Total Parenteral Nutrition (TPN) Nutrition maintained entirely by the administration of a nutritionally adequate solution through a catheter into a vena cava or by some other non gastrointestinal route. Used in cases of long-term comas or burns, or severe gastrointestinal syndromes.
Titer The concentration of a substance in solution, used as a measure of strength. More specifically, it refers to the concentration of antibodies against a specific antigen in the blood.
Thromboplastin Blood coagulation Factor III. This blood protein accelerates the clotting of blood by converting prothrombin into thrombin (in the presence of calcium ions). It is also called platelet tissue factor and thrombokinase.
Thrombocytopenia A decreased number of blood platelets, cells that are important for clotting.
Thrombin An enzyme that participates in blood clotting by converting fibrinogen to fibrin.
Tetanus Immune Globulin Immune globulin from human blood for use in treating persons not previously immunized against tetanus whose wound would indicate the need for tetanus prophylaxis. Human Tetanus Immune Globulin will produce fewer side effects than will tetanus antitoxin produced from horse serum.
Tetanus An acute, often fatal, disease that is characterized by the spasmodic contraction of voluntary muscles, especially those of the neck and jaw. It is caused by the toxin of the bacillus <em>Clostridium tetani</em> which typically infects the body through a deep wound. It is also called lockjaw.
T Lymphocytes / T Cell A type of lymphocyte that matures in the thymus gland and contributes to the cell-mediated immune response. These cells circulate between the blood and lymph nodes. There are three types of T cells: cytotoxic (killer), helper, and suppressor.
Surgical Oncology The surgical aspects of cancer including biopsy and surgical resection of tumors.
SubQ Immune Globulin See: Subcutaneous Immune Globulin
Subcutaneous Immune Globulin (SCIG) A sterile solution of concentrated antibodies extracted from healthy donors which is administered under the skin in several sites on the body. SCIG is used to treat disorders of the immune system or to boost immune response to serious illness, and to treat immunosuppressed recipients of bone marrow transplants.
Source Plasma Plasma that is supplied from donations through plasmapheresis, as opposed to recovered plasma which is obtained from donations of whole blood. Source plasma is collected at donation facilities specializing in plasmapheresis. Donor frequency averages 14 times per year in amounts smaller than typically donated at a blood drive. In a 25,000 liter pool of blood, there are approximately 15,600 donors. Small donations are pooled from repetitive donors. Derivatives of this plasma include albumin, IVIG, coagulation factors, and hyper-immunes. For comparison, see Recovered Plasma.
Somatropin for Injection Recombinant growth hormone (HGH). A protein-based peptide hormone which stimulates growth, cell reproduction and regeneration.
Solvent Detergent Treated A purification treatment useful in the sterilization of plasma-derived products such as coagulation factors and intravenous immune globulins. The solvent or detergent kills viruses by dissolving the lipid-envelope protective coating around them. Lipid-enveloped viruses include HIV, HBV, and HCV.
Serum The clear portion of any animal liquid separated from its more solid elements such as cells, especially the clear portion (blood serum) that separates during blood clotting.
Seropositive A positive result from a diagnostically useful serological test, indicating the presence of antibodies to the tested antigen.
Seronegative A negative result from a diagnostically useful serological test, indicating a lack of antibodies to the tested antigen.
SCIG See: Subcutaneous Immune Globulin
SARS Coronavirus The viral cause of severe acute respiratory distress syndrome (SARS), a respiratory illness that first emerged in 2002. It is characterized by high fever, cough, and difficulty breathing, and is highly contagious and lethal in some cases. The majority of reported cases have originated in China.
RSV See: Respiratory Syncytial Virus
Ristocetin Cofactor Unit (RCU) Unit of measurement for vonWillebrand factor, the medication used to treat vonWillebrand's Disease. It is a measure used to assess effectiveness and dosage. Brand name for this factor is Humate -P.
Rimabotulinumtoxin B for Injection Botulinum toxins are proteins produced by the bacterium Clostridium botulinum. RimabotulinumtoxinB is utilized in the treatment of cervical dystonia.
Rh Sensitization Also known as Rh Isoimmunization. In persons with an Rh-negative blood type, the development of antibodies to the Rh factor caused by exposure to Rh-positive blood. This condition is dangerous during pregnancy because of the Rh antibodies can be harmful to an Rh-positive fetus, possibly resulting in Hemolytic Disease of the Newborn. Rh sensitization after exposure is prevented through injection of Rh-antibodies, in the form of Rho(D) immune globulin, to clear the Rh-factor from the blood.
Respiratory Syncytial Virus (RSV) A virus that induces formation of syncytial masses in infected cell cultures. It is a major cause of respiratory disease in children.
Recovered Plasma Plasma that is supplied from donations of whole blood, as opposed to source plasma which is obtained through plasmapheresis. Recovered plasma is collected at blood drives. Donor frequency averages once or twice a year. Derivatives of this plasma include albumin, IVIG, coagulation factors, and hyper-immunes. In a 25,000 liter pool of blood, there are approximately 100,000 donors. For comparison, see Source Plasma.
Reconstitution Returning a substance previously altered for storage or preservation to its original state. For example, lyophilized (or powderized) IVIG's must be returned to a liquid state to be administered.
Recombinant Refers to biologically engineered blood coagulation factor Bio-Engineered Factor product. Contains no human component, lowering the risk of viral contamination. Recombinant Factor refers to genetically engineered or cloned FVIII, FIX, or FVII that is not derived from human plasma.
Recessive A genetic trait that is expressed only if both genes in a gene pair share the trait; will not be expressed if a dominant gene is present.
Radiation Oncology The treatment of cancer with therapeutic radiation. Radiation oncology is also called radiation therapy or radiotherapy.
Rabies Immune Globulin Rabies immune globulin is used along with rabies vaccine to prevent infection caused by the rabies virus. It works by giving your body the antibodies it needs to protect it against the rabies virus. This passive protection lasts long enough to protect your body until it can produce its own antibodies. Rabies immune globulin is given to people who have been exposed to an animal that may have the virus. This is used only in people who have never before received the rabies vaccine.
Rabies An acute, potentially fatal viral infection that attacks the central nervous system resulting in paralysis and eventually death. The rabies virus is carried primarily by wild animals, especially bats and raccoons. It is transmitted through infected saliva, usually through the bite of a rabid animal. The disease is characterized by increased salivation, abnormal behavior, eventual paralysis, and (when untreated) death. It is also called hydrophobia due to an infected person's inability to swallow water.
Pruritis Itching.
Prothrombin Complex Concentrate (PCC) PCC contains blood coagulation factors II, VII, IX and X, proteins C and S, and small amounts of activated coagulation factor.
Prothrombin Blood Coagulation Factor II, a protein in the blood that aids in clotting. Prothrombin is converted to thrombin through a series of reactions in response to bleeding. A chemical substance existing in circulating blood that, through the medium of thrombokinase, interacts with calcium salts to produce thrombin. It is formed and stored in the liver.
Protease Inhibitor Antiviral drugs that act by inhibiting the virus' protease enzyme, thereby preventing viral replication.
Prophylaxis Preventative treatment measures. In factor usage, it is the scheduled infusion of the necessary factor to maintain high levels in the blood stream and prevent most bleeds.
Product Shelf Life The time a product retains effectiveness after its date of manufacture.
Product Half Life The time that it takes for a medication's level and effectiveness in the body to reach half that of initial administration as it is metabolized or inactivated by the body. It is used as a measure of effectiveness in treatment and is important in determining the drug dosage amount and frequency.
Primary Immune Deficiency (PID) An immune deficiency arising from intrinsic defects in the cells of the immune system (as opposed to secondary, acquired deficiencies caused by infection). PID's are often caused by inherited genetic defects.
Primary Humoral Immunodeficiency See: Primary Immune Deficiency
PPF See: Plasma Protein Fraction
Platelets A small, disk-shaped blood cell with no nucleus that circulates in the blood and participates (together with fibrin) in forming blood clots. When an injury occurs, platelets adhere to one another and the edges of the injury to form a plug to cover the area. Platelets are derived from megakaryocytes. Also called a thrombocyte.
Plasmapheresis The process in which plasma is removed from donated blood and the remaining components, mostly red blood cells, are returned to the donor.
Plasma Protein Fraction (PPF) PPF, also known as human albumin serum, is a standard sterile preparation of serum albumin and globulin obtained by fractionating blood, serum, or albumin from healthy human donors. It is sterilized and tested. It is used as a blood volume expander, in the treatment of burns where albumin is lost rapidly, and in the treatment of hypoalbuminaemic patients with edema where diuretics have failed.
Plasma The clear, protein rich, fluid component of blood, containing dissolved solutes, and minus the red blood cells. Clotting factors are found in plasma. Note that plasma differs from serum in that it contains fibrin and other soluble clotting elements. It can be sterilized and administered intravenously.
PID See: Primary Immune Deficiency
Perinatal Transmission The transmission of a pathogen from mother to baby before, during, or after the birth process.
pepsin Pepsin is a digestive enzyme used by the stomach to digest food. It is also useful in the viral safety processing of plasma products when used in the presence of a lower pH solution.
PCC See: Prothrombin Complex Concentrate
Pathogen An agent of disease. The term pathogen most commonly refers to infectious organisms. These include bacteria such as staph, viruses such as HIV, and fungi such as yeast.
Pasteurization Process of heating liquids to destroy harmful or undesirable microorganisms.
Passive Immunity Immunity acquired through the transfer of antibodies from another person or animal. This may occur naturally, as from mother to fetus through the placenta or to infant through breast-feeding, or artificially through injection of immune globulin.
Parvovirus B19 A very small, non-enveloped virus that causes fifth disease, usually benign, non-febrile disease. Infection in immunocompromised patients may result in complications and may lead to death.
Oncology The field of medicine devoted to the study of cancer and tumors encompassing the physical, chemical, and biological properties. There are three primary disciplines: medical oncology, surgical oncology, and radiation oncology.
Oncologist A physician who specializes in the treatment of cancer. Some oncologists specialize in a particular type of cancer treatment. For example, a radiation oncologist specializes in treating cancer with radiation.
Neurologist A physician who specializes in the diagnosis and treatment of disorders of the nervous system.
NDC Number National Drug Code - The NDC number serves as a universal product identifier for human drugs. Each drug product listed under Section 510 of the Federal Food, Drug, and Cosmetic Act is assigned a unique, 10 digit, 3-segement number. The first segment identifies the manufacturer, the second and third identify the product and the package size. NDC Directory
Nanofiltration Nanofiltration is a viral removal process which passes the product to be filtered through an ultra-fine membrane filter for pathogen removal. Nanofiltration clears enveloped viruses, non-enveloped viruses, and virus-antibody complexes, with minimal or no effect on immunoglobulins. This process significantly reduces the risk of disease transmission.
Multiple Sclerosis An immune-mediated inflammatory disease that attacks the myelin sheath of axons (conductors for electrical impulses) in the central nervous system, thereby disrupting the function of the body controlled by the damaged cells.
MSDS Material Safety Data Sheet
Monoclonal A factor component antibody derived from a single cell, produced in large quantities for use against a specific antigen. Has the advantage of ultra-high purity, ensuring a low risk of viral contamination.
Menorrhagia Unusually heavy menstrual bleeding. Menorrhagia, especially in young women, is often a good indicator of the presence of a bleeding disorder such as von Willebrand's disease.
Medical Oncology The treatment of cancer with medicine, including chemotherapy.
Lyophilized A substance in a powder-like form that requires reconstitution prior to administration. Lyophilization is the process of rapidly freezing a substance at an extremely low temperature and then dehydrating in a high vacuum.
Lymphocytes White blood cells that fight infection and disease.
Lymphatic System The tissues and organs that produce, store and carry white blood cells that fight infections and disease. This system includes the bone marrow, spleen, thymus and lymph nodes and a network of thin tubes that carry lymph and white blood cells. These tubes branch, like blood vessels, into all the tissues of the body.
Lot Number A number assigned by a manufacturer to a specified quantity of product manufactured, necessary for tracking, testing and evaluation purposes. Also the Primary identifier for recalled products.
Kawasaki Disease An acute febrile disease of unknown cause that resembles scarlet fever. It affects primarily infants and children. The fever is present from one to three weeks. Named for Tomasaku Kawasaki, the contemporary Japanese pediatrician to first identify the disease. Treated with IVIG.
IVIG See: Intravenous Immune Globulin
ITP See: Idiopathic Thrombocytopenic Purpura.
Intravenous Immune Globulin (IVIG) Intravenous Immune Globulin - (IVIG or IGIV) A sterile solution of concentrated antibodies extracted from healthy donors which is administered into a vein. IVIG is used to treat disorders of the immune system or to boost immune response to serious illness, and to treat immuno suppressed recipients of bone marrow transplants.
Intravenous Within a blood vessel.
Intramuscular Immune Globulin (IMIG) This medication, administered within the muscle, is a sterile solution of antibodies extracted from health donors. It is used to provide protection (immunization) against Hepatitis A, measles, varicella and rubella infections. It is also used to boost the body's natural immune response in persons with immune system problems.
Intramuscular Within a muscle.
Intracerebral Hemorrhage (ICH) Also known as hemorrhagic stroke. ICH occurs when a blood vessel within the brain bursts, allowing blood to leak inside the brain. The sudden increase in pressure within the brain can cause damage to the brain cells. If the amount of blood increases rapidly, the sudden buildup in pressure can lead to severe neurological impairment, coma or death. The most common cause of intracerebral hemorrhage is high blood pressure (hypertension).
International Units (iu) The amount of a standardized preparation that is agreed upon as an international standard. These units are defined by the International Conference for Unification of Formulae. Usually set by the quantity of a biologically active substance required to produce a specific response. Most factors are sized and priced by iu.
Inhibitors Antibodies produced by the immune system in response to blood coagulation factor in the treatment of hemophilia. The inhibitor binds itself to the infused clotting factor, making it difficult (if not impossible) to obtain a factor level sufficient to control bleeding.
Infusion The therapeutic introduction of a fluid other than blood, such as saline solution, into a vein.
Influenza Vaccine Vaccine used to prevent the flu virus.
Indication A sign or circumstance that indicates the proper treatment of a disease. Also, the specific medical condition that a medication has been approved to treat.
Immunosuppression A condition in which a patient's immune system is prevented from functioning normally. This condition may result from disease or tolerance , or it may be intentionally induced artificially through drugs or radiation in order to prevent rejection of transplants or to control autoimmune diseases.
Immunoglobulin Subclasses of the gamma globulin fraction of plasma proteins, produced by plasma cells that have antibody functions, providing humoral immunity. All antibodies are immunoglobulins, but researchers have not yet determined whether immunoglobulins have antibody functions. Normally, globulins are present in human blood. It is used in passive immunization of immunosupressed patients exposed to various infectious agents. These are five classes of immunoglobulins: IgA, IgD, IgE, IgG, and IgM.
Immune Thrombocytopenic Purpura (ITP) Also known as idiopathic thrombocytopenic purpura. This is a condition of immune origin which results in lowered blood platelet count (thrombocytopenia) and excessive bruising (purpura). Platelets are destroyed by antibodies. Bruising results in purplish-looking areas of the skin and mucous membranes (such as the lining of the mouth) where bleeding has occurred as a result of the decreased number of platelets. Some cases of ITP are caused by drug use; others are associated with infection, pregnancy, or immune disorders. In about half of all cases, the cause is unknown (idiopathic). ITP is often related to HIV / AIDS. Also called purpura hemorrhagic or Welhof's Disease.
Immune System The complex group of organs and cells that defend the body against infection or disease.
Immune Response An integrated bodily response to a foreign antigen, neutralizing or eliminating the invading substance, and preventing damage. The response is mediated by lymphocytes which recognize the antigen as "non-self" through antibodies specific to each antigen.
Immune Globulin - Intravenous (IGIV) See: Intravenous Immune Globulin
Immune Deficiency A condition in which some component of the body's immune system is weak or absent, resulting in a decreased or compromised ability to respond to infection.
IMIG See: Immune Globulin-Intramuscular
IgM Immunoglobulin gamma M - The class of antibodies found circulating in the blood, and first to appear in initial response to exposure to an antigen (before IgG). IgM is highly efficient in stimulating complement activity. The human red cell isoantibodies which control the A, B, O blood groups are IgM antibodies. IgM has a very high molecular weight and is unable to cross the placental barrier to the fetus.
IGIV See: Intravenous Immune Globulin
IgG Immunoglobulin G - The principal immunoglobulin in human serum. It is the major antibody for antitoxins, viruses, bacteria, fungi, and other foreign particles. IgG facilitates the phagocytic destruction of these antigens by agglutinating them, opsonizing them or by activating the complement system against them. IgG may be administered to provide temporary resistance to hepatitis or other diseases. IgG has a low molecular weight, and is thus the only gamma globulin able to cross the placental barrier, providing immune protection to the fetus before birth.
IgE Immunoglobulin E - IgE plays a major role in allergic reactions. It is produced in the lungs, skin, and mucous membranes, and attaches to mast cells in the respiratory and intestinal tract. About half of the patients with allergic diseases have increased IgE levels.
IgD Immunoglobulin D. The class of antibodies found only on the surface of B lymphocytes, which function as antigen receptors to initiate differentiation of B-cells into plasma cells.
IgA Immunoglobulin A - The principal antibody type in external bodily secretions such as saliva, tears, sweat, respiratory and intestinal mucin, and breast milk. IgA provides local immunity against infections in the gut and respiratory tracts by preventing the attachment of viruses and bacteria to mucosal epithelial surfaces. Its presence in colostrum helps to protect a newborn from infection.
Idiopathic Without a known cause.
Hypogammaglobulinemia Deficiency of immunoglobulins, resulting in a weakened immune system and an increased susceptibility to infection. Treatment includes administration of intravenous immune globulin (IVIG).
Hypersensitivity An abnormal, exaggerated immune response that may be either immediate (due to antibodies of the IgE class, e.g. an allergy to ragweed) or delayed (due to cell-mediated immunity, e.g. a transplant rejection). It may result in tissue damage.
Hyper-immune In reference to plasma, it refers to a plasma product with a very high titer of a specific antibody. Administration of this plasma results in the immune system being much more reactive to the specific antigen than it normally would be.
Humoral Immunity Immune defense in which antibodies are produced by B-lymphocytes and circulate as soluble proteins in blood in lymph (or humors0. This is in comparison to cell-medicated immunity in which the T-cells come in close proximity to their victim cells). Humoral immunity is acquired; antibodies are produced by B- cells only in response to specific antigens.
HIV Human Immunodeficiency Virus. Acute infectious manifestations, neurological disorders, and immunologic abnormalities linked to the acquired immunodeficiency syndrome (AIDS).
High-Titer Immune Globulin See: Hyper-Immune
Hereditary Angioedema (HAE) Deficiency of C1 esterase inhibitor, which causes attacks of rapid swelling in the hands, feet, limbs, face, intestinal tract, larynx or trachea. Attacks which obstruct airways can be fatal.
Hepatitis Inflammation of the liver. It may be caused by a variety of agents, including viral infections, bacterial invasion, and physical or chemical agents. It is characterized by jaundice, fever, liver enlargement, and abdominal pain.
Hemostasis The process which stops bleeding at an injury's site.
Hemophilia B Disease in which the blood does not clot properly due to a deficiency of blood coagulation Factor IX. Also known as Christmas Disease. Treated by administering Factor IX. The disease is an X-linked trait and occurs mostly in males. It is so named because the first patient with the disease to be studied in great detail was named Christmas.
Hemophilia A Disease in which the blood does not clot properly due to a deficiency of blood coagulation Factor VIII. Treated by administering Factor VIII. The disease is an X-linked trait and occurs mostly in males.
Hemophilia Disease in which the blood does not clot properly. Any of several hereditary disorders in which blood takes an abnormally long time to coagulate due to a deficiency or abnormality in one of the blood coagulation factors. It is a recessive, X-linked trait which manifests almost exclusively in males. Females can carry the gene without having the disease; each son of a female carrier has a 50% chance of receiving the gene and having hemophilia. There is no cure for hemophilia. It is treated through the administration of the deficient factor.
Hemolytic Disease of the Newborn (HDN) This is a potentially severe condition affecting newborns. If a mother has developed antibodies to her fetus' blood type, the mother's blood attacks the fetus' blood, causing the red blood cells to burst. The baby is born anemic, jaundiced, and with an enlarged liver and spleen. This condition is most easily prevented by careful prenatal care in which the mother's blood is prevented from forming the antibodies against the fetus' blood.
Hemolytic Anemia A low red blood cell count resulting from any medical condition which causes red blood cells to burst.
Hemolysis The rupture of red blood cells.
Hemoglobin The component of red blood cells that carries oxygen from the lungs to the tissues of the body.
Hemin Injection Hemin is an iron-containing porphyrin used in the management of porphyria attacks, particularly in acute intermittent porphyria.
Hematuria Presence of blood in the urine.
Hematology The study of blood-producing tissues, and diagnosis and treatment of disorders of blood and blood-producing tissues.
Hemarthrosis Accumulation of blood in a joint or joint cavity; extreme swelling often results. This condition is a common symptom of hemophilia and can lead to pain and stiffness of the joints. Occurrence of hemarthrosis in hemophiliacs may be lowered through prophylactic dosage of the appropriate coagulation factor.
Heat Treated Viral inactivation process that uses heat up to 80 degrees C.
HAE See: Hereditary Angioedema
Guillian - Barre Syndrome Also known as acute inflammatory polyneuropathy or Landry's ascending paralysis, this rare neurological syndrome affects 1-2 people per 100,000. Symptoms of this ascending paralysis begin with loss of function in the extremities that progresses throughout the body. Often respiratory muscles are affected, resulting in respiratory failure. Muscle weakness and pain in the hips, thighs and back are common. In severe cases, complete paralysis may be experienced. Recovery may be lengthy (sometimes more than a year), and may be partial or complete. The fact that this syndrome often follows an acute viral or bacterial infection suggests that it is an immune-regulated antibody response to an infection which also mistakenly affects the myelin sheaths of the nerves, resulting in demyelination of the peripheral nerves. IVIG is commonly used to treat Guillian Barre patients with rapidly progressing paralysis.
Growth Hormone Deficiency A deficiency of the regulating growth hormone. Growth hormone is produced in the pituitary gland, and causes for its deficiency range from congenital conditions such as gene mutation and congenital malformations of the pituitary to autoimmune inflammation and stroke.
Graft-v-Host Disease (GVHD) A reaction of donated bone marrow against the recipient patient's own tissue.
Globulin Simple proteins that are found in the blood serum, which contain molecules central to the immune system function.
Glatiramer Acetate Injection An immunomodulator used to treat multiple sclerosis, it is thought to act as a decoy, diverting the autoimmune response against myelin. Glatiramer Acetate is administered by subcutaneous injection.
Gamma Globulin One of the proteins in blood serum that contains antibodies. Passive immunizing agents obtained from pooled human plasma.
Fractionation Process by which plasma is broken down into its various components.
Fibrinolysis The dissolution of a blood clot. The process breaks down the fibrin polymers of the clot and prevents more from forming.
Fibrinogen A protein in blood plasma that is essential for blood clotting. It is converted to fibrin, the protein which constitutes the bulk of a blood clot, through the action of thrombin in the presence of calcium ions. Also called blood coagulation Factor I.
Fibrin The insoluble protein in blood plasma that constitutes the bulk of a blood clot. It forms an interlacing, fibrous network.
Febrile Feverish.
Factor X Stuart-Prower Factor.
Factor VIII Blood coagulation Factor VIII, or Anti-Hemophilic Factor (AHF). A protein substance in blood plasma that is essential in the blood clotting process. Hemophilia A, the most common form of blood clotting disorder, is caused by a deficiency of Factor VIII. Human plasma can be fractioned such that Factor VIII remains. The resulting plasma, containing Factor VIII, can be given to hemophiliacs to temporarily correct the bleeding tendency.
Factor VII Blood coagulation Factor VII, also known as proconvertin, or serum prothrombin conversion accelerator. A protein substance in blood plasma that is essential in the blood clotting process. It is formed in the kidney under the influence of vitamin K and may be deficient due to a hereditary disorder. In clotting, it forms a complex with tissue thromboplastin and calcium to activate prothrombokinase, thus acting to accelerate the conversion of prothrombin to thrombin.
Factor IX Blood coagulation Factor IX, also known as Christmas Factor (CF) and Plasma Thromboplastin Component (PTC). In the blood clotting process, Factor IX activates thromboplastin. Deficiency results in Hemophilia B, or Christmas Disease.
Factor IV Calcium ions.
Factor III Thromboplastin (Tissue Factor).
Factor II Prothrombin, one of several proteins found in the blood that aids in coagulation.
Factor I Fibrinogen, one of several proteins found in the blood that aid in coagulation or clotting. It is converted to fibrin, the protein which constitutes the bulk of a blood clot, through the action of thrombin in the presence of calcium ions.
Etiology The cause of a disease.
Erythroblastosis fetalis Also known as Hemolytic Disease of the Newborn (HDN). This is a potentially severe condition affecting newborns. If a mother has developed antibodies to her fetus' blood type, the mother's blood attacks the fetus' blood, causing the red blood cells to burst. The baby is born anemic, jaundiced, and with an enlarged liver and spleen. This condition is most easily prevented by careful prenatal care in which the mother's blood is prevented from forming the antibodies against the fetus' blood.
Epistaxis Frequent and prolonged nosebleeds. Commonly a symptom of bleeding disorders, especially von Willebrand's Disease.
Efficacy The extent to which a particular treatment produces the desired effect.
Disseminated Intravascular Coagulation (DIC) A pathological condition in which blood to coagulates throughout the whole body. Consequently, the body becomes depleted of platelets and coagulation factors, and generalized bleeding occurs. This condition results from one of a number of possible underlying primary illnesses. DIC is associated with sepsis (especially from a gram-negative infection), obstetric complications, malignant cancers, trauma, and other conditions that result in the release of chemicals into the blood, instigating blood coagulation. Treatment requires that the primary illness be addressed. In some cases, heparins or anti-thrombin III may also be administered.
Diluent An agent that is used to dilute the substance or solution to which it is added.
Cytomegalovirus Prophylaxis Preventative treatment in CMV-positive patients. Treatment with IVIG to control CMV, allowing the body's own immune system to deal with other infections.
Cytomegalovirus (CMV) One of a group of species-specific herpes viruses. They inhabit salivary glands and cause the enlargement of epithelial cells. Though found in a large portion of the human population, it causes disease only in utero (resulting in abortion, stillbirth, or various congenital defects), but can also be opportunistic in immune-deficient patients. People who have been exposed to the virus will remain cytomegalovirus IgG positive.
Cryoprecipitate A blood plasma derivative product that is rich in coagulation factor VIII and also contains fibrinogen, factor IX, factor VIIa, and von Willebrand's factor. The preparation process involves warming frozen plasma to separate out the desired portion.
Creutzfeldt-Jakob Disease (CJD) A disease that affects the central nervous system. It is a spongiform encephalopathy, meaning that as the disease progresses, it forms holes in the brain. It results in rapid and progressive loss of muscle control, dementia, memory loss, hallucinations, speech impairments, and difficulty walking and talking. There is no cure for CJD, and it is always fatal, usually within a year of onset. CJD is believed to be caused by a type of prion, a proteinaceous infectious particle that is responsible for several diseases of the central nervous system. CJD affects adults, especially older adults, with an average onset of 50 years of age. Variant Creutzfeldt-Jakob Disease (vCJD) is caused by a similar prion and has related symptoms, but affects a much younger population, with an average age of onset in the mid 20's. Bovine Spongiform Encephalopathy, or mad cow disease, is thought to be caused by a prion similar to that of CJD and vCJD. No connection has been found between mad cow disease and CJD, but it has been linked to cases of vCJD in which the patients ate infected cow meat.
Coumarin A chemical compound which can be extracted from plants. This compoud is a component of the anticoagulant coumadin, which is frequently used to inhibit blood clotting.
Contraindication Any symptom or circumstance indicating the inappropriateness of a form of treatment that would otherwise be advisable.
Congenital Describing a condition present at birth.
Complement System The complement system is a group of proteins in the blood with immune functions. Through a complex cascade of biochemical interactions, complement can act to protect the body in several ways: complement can cause a foreign cell to burst open and die; it can mark a foreign cell for destruction by a macrophage; and it can result in inflammation and stimulation of the B-cell-medicated immune response. Complement remains inactive in the blood until activated through one of several pathways. Deficiency of any of the more than 35 proteins involved the complement system may result in an increased susceptibility to infection. Deficiency of specific complement proteins has been linked to certain autoimmune diseases, increased susceptibility to bacterial infections, and certain hereditary conditions.
Combined Immunodeficiency Immunodeficiency in which the patient has a deficiency of both B-lymphocytes and T-lymphocytes.
Coagulation Factor The factors that participate in the blood coagulation process. Over 30 types of cells and substances contribute to blood clotting. There are a large number of terms that have been applied to coagulation factors, but the generally accepted terms, as well as Roman Numerals , are included here.
Coagulation Clotting of the blood. The process in which blood is changed into a jelly-like substance to seal or plug an injured blood vessel. Hemophilia patients have less of the necessary blood coagulation factors necessary to seal a wound. Coagulation of blood occurs in two pathways - intrinsic (activated by negatively charged substance, such as a collagen at the site of a wound) or extrinsic (activated by tissue thromboplastin within a tissue). These two pathways differ in the beginning stages, but share a common pathway for the final clot formation.
CMV See: Cytomegalovirus
Clotting Factors The proteins that are needed to make blood clot.
CLL See: Chronic Lymphocytic Leukemia
Chronic Lymphocytic Leukemia (CLL) Chronic leukemia marked by an abnormal increase in B-lymphocytes. It is characterized by slow onset and progression of symptoms, including anemia, pallor, fatigue, appetite loss, lowered granulocyte, platelet counts, gamma globulin counts, and enlargement of lymph nodes, liver, and spleen. More common in older men. May be treated with IVIGs.
Christmas Factor Blood coagulation Factor IX. Also known as Plasma Thromboplastin Component (PTC). In the blood clotting process, CF activates thromboplastin. Deficiency results in Hemophilia B, or Christmas Disease.
Christmas Disease Disease in which the blood does not clot properly, also called Hemophilia B. It is caused by a deficiency of blood coagulation Factor IX. The disease is an X-linked trait and occurs in males. It is so named because the first patient with the disease to be studied in great detail was named Christmas.
Chemotherapy Drug therapy for the treatment of cancer.
Cervical Dystonia A syndrome of involuntary sustained of spasmodic muscle contractions of the head and neck, resulting in abnormal posturing or jerky movements of the head. Cervical dystonia is sometimes treated with botulinum toxin injections into the affected muscles.
Cell-Mediated Immunity Immune defense in which T-lymphocytes come in close proximity with their victim cells, as opposed to humoral immunity which is provided by the secretion of antibodies into the blood or lymph (humors) by B-lymphocytes.
Cancer A term used to describe diseases in which abnormal cells divide without control. Cancer cells can invade nearby tissues and can spread through the bloodstream and lymphatic system to other parts of the body.
C1-Esterase Inhibitor A protease inhibitor which prevents spontaneous activation of the complement system, as well as parts of the lectin pathway, and proteases of the fibrinolytic, clotting, and kinin pathways. A deficiency of C1-esterase inhibitor causes Hereditary Angioedema.
Bone Marrow Transplant (BMT) A medical procedure in which bone marrow is removed from one person and transplanted in another in order to stimulate production of blood cells. It is used to treat malignancies, certain forms of anemia, and immune deficiencies. IVIG may be used during the course of BMT treatment.
Bone Marrow The soft, spongy tissue in the center of large bones that produces white blood cells, red blood cells and platelets.
Bioavailability The proportion of an administered drug that enters general circulation and is available for use by the body. Depending on the drug's method of action and purpose, intramuscular and subcutaneous injections may result in a lower bioavailability of the drug than would an intravenous injection.
Bacteremia The presence of viable bacteria in the blood.
B-Lymphocytes / B-Cells The type of lymphocyte that is responsible for the humoral immune response. It matures in the bone marrow and migrates to the tissues of the lymph system where it waits to be stimulated by a foreign antigen to produce antibodies. B-cells are antigen-specific, meaning that they only respond to one antigen. Because the spleen and lymph nodes have a large volume of blood passing through them, these organs are rich in B-cells, increasing the lymphocytes' subsequent exposure to new antigens.
Autoimmune Disease A disease occurring when an individual's immune system attacks the body's own tissues or extracellular proteins. This disease results from a reduction in the body's tolerance to the antigens located on its own cells. For example, multiple sclerosis results from the body's own antibodies attacking the myelin sheath that covers nerve cells. Researchers have begun clinical studies using IVIG to treat certain autoimmune diseases.
Antithrombin III Deficiency A rare hereditary disorder, often presenting as frequent venous thrombosis or pulmonary embolism. Patients are treated with anticoagulants and antithrombin concentrates.
Antithrombin III A small protein molecule which inactivates several enzymes in the coagulation cascade.
Antigen-Specificity The property B- and T-lymphocytes gain through an initial exposure to an antigen to recognize that specific antigen if it again enters the body. Antigen-specificity allows the body to more quickly mediate an immune response to each subsequent exposure to that specific antigen. This property is the basis of immunization.
Antigen-Antibody Reaction The binding of a specific antigen with its corresponding antibody. It may result in agglutination, precipitation, or increased susceptibility to phagocytosis.
Antigen In broad usage, any substance or entity which the body recognizes as foreign and induces the production of antibodies to neutralize or destroy the antigen. Antigens include toxins, bacteria, foreign blood cells, and the cells of transplanted organs. More specifically, the antigen is the protein marker on the surface of a cell that marks it as "self" or "non-self." The antigen identifies the type of cell (skin, kidney, foreign substance). Autoantigens are markers on the body's own cells; markers on all other antigens are called foreign antigens.
Antibody An immune, or protective, protein secreted into the blood in response to foreign substances, called antigens, being introduced to the body. It combats these viruses, bacteria, and parasites, and some transplanted organs, by binding to the foreign substance. Once bound, the antibody leads to the neutralization or destruction of the antigen, providing protection from most common infections. Antibodies neutralize or destroy antigens in several ways. They can directly lyse the antigen, neutralize toxins released by bacteria, opsonize the antigen (form a complex to stimulate phagocytosis), promote antigen clumping (agglutination), or prevent the antigen from adhering to host cells. Antibodies are Y-shaped immunoglobulins produced by B- lymphocytes. Their synthesis is induced by specific antigens. They combine with the specific antigens which stimulated their production, and not with unrelated antigens. Antibodies are responsible for humoral immunity. Inhibitors that occur in response to replacement therapies for Hemophilia A are antibodies to Factor VIII.
Anti-Hemophilic Factor (AHF) Blood coagulation Factor VIII, a protein substance in blood plasma that is essential in the blood clotting process. Hemophilia A, the most common form of blood clotting disorder, is caused by a deficiency of AHF. AHF can be separated out of human plasma and given to hemophiliacs to temporarily correct the bleeding tendency.
Anaphylaxis An unusually severe allergic reaction, usually to food, drug, or insect bite. It occurs between an allergic antigen, and IgE bound to mast cells, stimulating the sudden release of immunological mediators locally and throughout the body. Initial symptoms appear within minutes, but recurrence may follow hours later. Hypersensitivity must have been induced in the individual in a prior exposure to the allergen for an anaphylactic reaction to occur. It is the initial exposure that results in the IgE binding to mast cells. The reaction can result in cardiovascular shock and death. It is categorized as local or systemic. Local anaphylactic reactions include hay fever, hives, and allergic gastroenteritis. Systemic anaphylaxis, which produces peripheral vasodilation, brochospasm, and laryngeal edema, can be life threatening.
Albumin One of a group of simple proteins made by the liver. Serum albumin is the main protein found in the blood (about 60 percent of the total). It is responsible for much of the plasma colloidal osmotic pressure and serves as a transport protein. Human albumin USP is administered intravenously to restore blood volume following shock, trauma, surgery, and burns.
AIDS See: Acquired Immunodeficiency Syndrome
AHF See: Anti-Hemophilic Factor
Agglutination The clumping together of red blood cells or bacteria; a type of antigen-antibody reaction in which a solid antigen clumps together with a soluble antibody. This physiological property is useful in laboratory testing.
Agammaglobulinemia A nearly total absence of immunoglobulins resulting in the loss of ability to produce immune antibodies.
Acute Intermittent Porphyria A rare autosomal dominant metabolic disorder characterized by a deficiency of the enzyme porphyobilinogen deaminase, which affects the production of the oxygen-binding prosthetic group of hemoglobin. Major symptoms include abdominal pain or cramping and neuropathies, sometimes leading to misdiagnosis as a psychiatric disorder.
Acute Describing a medical condition which is marked by rapid onset, severe symptoms, and short course.
Acquired Immunodeficiency Syndrome (AIDS) An acquired defect or cellular immunity associated with infection by the human immunodeficiency virus (HIV). AIDS increases susceptibility to opportunistic infections. The CDC lists numerous infections and cancers that, in the presence of HIV infection, constitute an AIDS diagnosis.