Glossary

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A

Accelerator Coagulation factors (FV, FVIII) that increase the rate of clotting within the coagulation cascade and are degraded after the process
Acquired related to diseases or disorders developed during life rather than inherited
Acquired Immune Deficiency Syndrome (AIDS) a disease that attacks and destroys the body's immune system, leaving patient abnormally vulnerable to infections and diseases
Activated partial thromboplastin time (aPTT) a blood test that determines how long it takes the body to form a clot
Adsorption taking up and holding on to the surface; a chromatographic resin attracts and holds on to a specific substance present in a solution that passes over its surface
Afibrinogenemia absence of fibrinogen, usually due to a congenital defect
Aggregate a collection
AHF abbreviation for anti hemophilic factor
AH Sepharose aminohexyl Sepharose, a starch-like material employed in a chromatography column; used for example in the processing of Monoclate® P to concentrate factor VIII:C and remove mouse protein
Anemia a condition in which the blood is deficient in red cells, in hemoglobin, or in total volume
Angiohemophilia alternative, largely outdated term for von Willebrand disease
Anticoagulant a substance that serves to prevent blood from clotting, such as coumarin or heparin
Antifibrinolytics local clotting agents that help protect blood clots and hold them in place
Antigen a substance against which the body produces antibodies (may be a virus, a bacterial infection or an infused or ingested protein)
Antihemophilic factor A factor VIII
Antihemophilic factor B factor IX, Christmas factor
Arthropathy degenerative joint disease
AT III antithrombin III
Atrophy wasting away, degeneration, or loss of size or function of tissue organ or limb
Autosomal inheritance inheritance independent of the sex chromosome; an autosomal disease can be inherited by both the male and female offspring

B

Batch refeed process a manufacturing process used in recombinant production
Bethesda unit (BU) a measure used to quantitate the concentration of inhibiting antibodies in the blood; 1 BU decreases the activity by 50%
Bleeding disorder a distinct condition in which a person's body cannot properly develop a clot
Bleeding time (BT) a test that screens the overall adequacy of platelet plug formation, measured as the time a standard skin cut takes to stop bleeding
Blood clot a semi-solid, spongy mass composed of fibrin strands in which the formed elements of blood are enmeshed
Blood vessel tubes that carry blood to the body's organs and extremities
B19 parvovirus a non-enveloped human virus; the cause of "fifth disease," a mild disease in children. May cause spontaneous abortions, or serious congenital disease of infants born to affected women; can affect the bone marrow, leading to anemia in patients with lowered immune defense; may also cause arthritis
BT See Bleeding time
BU See Bethesda unit
BVDV abbreviation for bovine viral diarrhea virus; a model virus for Hepatitis C used to test the viral reduction capacity of the manufacturing process of plasma-derived products

C

Ca++ calcium ion, factor IV in the coagulation cascade
CCID50 abbreviation of cell culture infectious dose 50%; in vitro assay for the estimation of viral reduction by viral inactivation/elimination procedures
CHO Chinese Hamster Ovary cell line, used for the manufacturing of some recombinant coagulation factors
Christmas factor factor IX, antihemophilic factor B
Chromosome a structure within a cell's nucleus that carries genes with their genetic information; a normal human cell has 46 chromosomes, 2x22 autosomes and 2x1 sex-chromosomes
CJD Creutzfeldt-Jakob disease
Classic hemophilia alternative, largely outdated term for hemophilia A
Clone a colony of genetically identical cells derived from a single cell
Clot see blood clot
Clotting disorder an abnormality in the process of blood clotting
Clotting factors plasma proteins involved in the clotting of blood; Coagulation factors
Coagulation clotting of blood
Coagulation cascade an extremely complex process requiring the coordinated interaction of endothelial cells, platelets and plasma proteins, leading to well-ordered activation deactivation between coagulation factors to retain the integrity of the blood vessel system and promote healing
Coagulation disorder see clotting disorder
Coagulation factor see clotting factor
Cofactor a protein, such as factor V or VIII, that is required for the normal clotting process to proceed, but is not directly involved in the coagulation cascade; a catalyst or accelerator
Collagen the protein of the white fibers of the skin, tendon, bone, cartilage and all other connective tissue; collagen in tissue surrounding blood vessels causes platelets to adhere to the site of injury of a blood vessel
Complementary DNA cDNA, DNA synthesized using mRNA as a template, cDNA is of value in rDNA technology
Congenital present at birth, inherited
Consumption coagulopathy: a decrease of coagulability of the blood, caused by excessive consumption of available clotting factors in blood
Contact factor factor XII
Coumarin a class of oral anticoagulants taken to inhibit the production of the vitamin K-, liver-dependent factors II, VII, IX, and X, and to minimize the risk of thrombosis
Covalent binding firm joining of two molecules
Cryoprecipitation the process used for separating VWF and factor VIII from other clotting factors in blood; in cryoprecipitation, the frozen plasma is thawed at 4°C and the clear solution containing most of the smaller plasma proteins is centrifuged to separate the cloudy portion, which contains VWF, factor VIII and fibrinogen
Culture medium the fluid in which micro-organisms or cells are grown

D

Detergent a cleaning agent that works by dissolving a contaminant; a substance soluble in both oil and water; when referring to the preparation of a plasma product, a substance that causes viral inactivation by dissolving the lipid membrane of enveloped viruses
Diafiltration a purification procedure in which large quantities of liquid pass through a semipermeable membrane, combining the techniques of dialysis and filtration; the desired material is not dialyzable
Dialysis a purification procedure in which an impure solution is passed through a semipermeable membrane that will allow removal of undesired components
DIC a serious complication of sereral diseases, which is characterized by a disturbance of the balance between pro- and anticoagulatory factors, leading to an abnormal coagulability
DNA desoxyribonucleic acid; the nuclear carrier of genetic information for all organisms except RNA viruses
Dry heat treatment in the processing of a plasma product, heating of lyophilized final material in the dry state, to achieve viral inactivation
Dysfibrinogenemia abnormality of the fibrinogen-molecule, sometimes caused by a congenital defect, in which the fibrinogen cannot function to form a normal clot
Dysprothrombinemia a bleeding disorder that slows the blood clotting process, leading to prolonged bleeding following an injury, surgery or having a tooth pulled; also called factor II deficiency or prothrombin deficiency

E

Ecchymosis a form of bleeding beneath the skin
Elastin a connective tissue protein present in the skin
Eluate a solution containing substance dissociated from a chromatography column
Elution the process of dissociating substances adsorbed onto a chromatography column
Endothelial cell the type of cell that lines the inside of blood vessels and some body cavities
Endothelium the layer of endothelial cells that lines the cavities of the heart, blood and lymph vessels and the serous cavities of the body
Enzyme a protein that catalyzes biochemical reactions without being altered, but is used up
Epistaxis bleeding from the nose
Extrinsic pathway when referring to blood coagulation, the pathway of reactions that is initiated by injury to tissue

F

Factor deficiency a rare disorder identified by the particular deficient or missing clotting factor in a person's body. Rare disorders include factor I, II, V, VII, VIII, IX, X, XI, XII, and XIII deficiencies
Fibrin an insoluble protein which is the end product of the coagulation reactions and which gives strength to a blood clot
Fibrin stabilizing factor factor XIII
Fibrinogen factor I, the precursor of fibrin
Fibrinolysis breaking down of a fibrin clot
Fibronectin a glycoprotein; some fibronectins cross-link collagen in connective tissue, others are involved in the aggregation of platelets
Fitzgerald factor high molecular-weight kininogen, HMWK
Fletcher factor prekallikrein
Fresh frozen plasma (FFP) plasma that has been separated from blood and frozen, soon after the collection from a donor; contains all clotting factors in unpurified, diluted and potentially infectious form
F I Fibrinogen
F II Prothrombin
F III Tissue factor; tissue thromboplastin
F IV Ca++
F V Proaccelerin
F VII Proconvertin
F VIII antihemophilic factor A
F IX Christmas factor or antihemophilic factor B
F X Stewart-Prower factor
F XI Plasma thromboplastin antecedent
F XII Hageman factor
F XIII Fibrin stabilizing factor

G

Gene a segment of DNA that contains all the information required for the biosynthesis of a protein
Genome One haploid set of chromosomes with the genes they contain
Glycoprotein a substance composed of carbohydrate such as sugar (glyco-) and protein portions

H

Hageman factor factor XII
Half-life the time it takes for half of the amount of a substance to be metabolized or leave the bloodstream, or for the activity of an enzyme to be reduced 50%
HAV hepatitis A virus
HbSAg hepatitis B surface antigen
HBV hepatitis B virus
HCV hepatitis C virus
Hemarthrosis bleeding into, or blood within, a joint
Hematologist a physician who specializes in blood and bleeding disorders
Hematoma bleeding into or collection of blood in tissue or muscle
Hematuria bleeding into, or blood within, urine
Hemoglobin an iron-containing respiratory pigment of vertebrate red blood cells that functions primarily in the transport of oxygen from the lungs to the tissue of the body
Hemolysis abnormal destruction of red blood cells (i.e by isoagglutinines)
Hemophilia A an X-chromosome linked, recessive disorder of blood coagulation caused by a deficiency or abnormality of factor VIII
Hemophilia B an X-chromosome linked, recessive disorder of blood coagulation caused by a deficiency or disorder of factor IX
Hemophilia Treatment Center (HTC) a federally funded hospital that specializes in treating patients with bleeding disorders. Each HTC has at least a hematologist, a nurse, a social worker and a physical therapist working as a team to deliver comprehensive care and support to patients with bleeding disorders and their families
Hemorrhage bleeding
Hemorrhagic pertaining to, or characterized by, bleeding
Hemostasis stopping of bleeding, blood coagulation
Hemostatic pertaining to the stopping of bleeding
Heparin an anticoagulant
Hepatitis infection and/or inflammation of the liver
Hepatitis A hepatitis caused by hepatitis A virus
Hepatitis B hepatitis caused by hepatitis B virus
Hepatitis C hepatitis caused by hepatitis C virus
Herpes simplex virus (HSV) Type I a virus employed as a model virus for large and enveloped DNA-viruses to test the viral reduction capacity of the manufacturing process of a plasma-derived product; the cause of fever blisters
High-molecular-weight kininogen (HMWK) a protein involved in the activation of factor XII to XIIa, and factor XI to XIa
HIV human immunodeficiency virus, the cause of AIDS
HMWK high-molecular-weight kininogen
Human immunodeficiency virus HIV, the cause of AIDS; there is more than one type of HIV, such as HIV-1 and HIV-2
Hypofibrinogenemia lower-than-normal blood concentration of fibrinogen; may be congenital or acquired

I

Idiopathic thrombocytopenic purpura (ITP) purpura associated with a decreased number of blood platelets, named when its cause was unknown (idiopathic) but now thought to be an autoimmune disorder
Immune system a system that protects the body from foreign substances, cells, and tissues by producing the immune response, the thymus, spleen, lymph nodes, special deposits of lymphoid tissue (as in the gastrointestinal tract and bone marrow), lymphocytes including the B cells and T cells, and antibodies
Immunodepletion removal of antibodies against inhibitors — eg, against F VIII — by immunoadsorbent chromatography of the blood of patients with inhibitors
Immunomodulation modifying or altering the immune response
Immunosuppression prevention or diminution of the immune response
Infusion intravenous administration of a greater volume of solution over a period of time
Inherited bleeding disorder a bleeding disorder received from a parent or ancestor by genetic transmission
Inhibitor 1) an agent that slows or interferes with a chemical reaction 2) a substance that reduces the activity of another substance (such as an enzyme)
Intracranial within the skull
Intramuscular within the muscle
Intravenous within a vein
Intrinsic pathway when applied to blood coagulation, the pathway of reactions that requires only factors present in plasma
In vitro literally, in glass; observable in a test tube
In vivo literally, in life; occurring in the body
Ion a molecule or atom carrying an electrical charge
Ischemia insufficient blood flow through the tissue leading to lack of oxygen
Isoagglutinin an antibody that causes red blood cells having a specific type of antigens to agglutinate (clump); anti-A antibody is an isoagglutinin which agglutinates type A red blood cells
ITI immune tolerance induction
ITT immune tolerance therapy. The process of eliminating inhibitory antibodies against FVIII or FIX therapy
IU International Unit, an internationally agreed on unit of measure used, for example, to measure activity of factor VIII:C

K

Kallikrein an enzyme required for the activation of factor XII to Xlla
Kinin one of a group of peptides that causes vasodilatation and increases vascular permeability
Kininogen the precursor of the kinins

L

Local clotting agent treatment that helps to protect blood clots and keep them in place to stop bleeding
Log in reporting reduction or inactivation of virus accomplished by a step in the manufacturing process, the logarithm of the number of virus infectious doses; thus, a 4 log reduction factor is a reduction factor of 10,000 in the base 10 logarithm system
Logarithm a mathematical device for simplifying calculations; the power to which the base of the logarithm system must be raised to equal a given number, for example, the logarithm of 100 in the base 10 system is 2 (102 = 10C)

M

MAb/MAbs monoclonal antibody/monoclonal antibodies
Macrophage large mononuclear phagocytic cell, tissue macrophages are derived from monocytes in the blood stream
Menorrhagia prolonged and/or heavy menstrual bleeding
Monoclonal relating to a single cell line, Le. a line of identical cells originating from a single cultured cell
Monoclonal antibody a specific antibody obtained from a cell culture that was started with a single B-cell
Monocyte a mononuclear phagocytic leukocyte in the blood: monocytes can develop into macrophages in the tissue
Monomer a single molecule of a compound of relatively low molecular weight, which tends to bind to longer chains (polymer)
Multimer a compound consisting of a number of identical smaller compounds (monomers)
Murine pertaining to rodents, particularly mice
Mutation a spontaneous change in the structure of a gene, leading to abnormal synthesis which subsequently night lead to a disease

N

Nanogram one-billionth of a gram; lo-9 g
Necrosis death, usually applied to tissue
ng nanogram, one-billionth of a gram
nvCJD New variant Creutzfeldt-Jakob disease

O

Oedema abnormal tissue swelling due to accumulation of fluid in body tissues
Oozing capillary bleeding

P

Pasteurization a unique method of pathogen inactivation introduced for FVIII-products by CSL Behring in the late 1970s. A very effective method to inactivate enveloped a non-enveloped viruses, but gentle enough to assure high product quality
Paresis partial or complete paralysis of muscles
Partial thromboplastin time (PTT) a blood test that screens for the overall response of the intrinsic pathway of coagulation
Parvovirus B19 see B19 parvovirus
PCC prothrombin complex concentrate
Petechia tiny red dot in the skin due to pinpoint bleeding, mainly caused by platelet abnormalities
PK pharmacokinetic
Phospholipid (PL) a phospholipids, coming mainly from activated platelets, required for the activation of factor X to Xa, and prothrombin to thrombin
Plasma the pale yellow fluid portion of whole blood that consists of water and its dissolved constituents including proteins (as albumin, fibrinogen, and globulins), electrolytes (as sodium and chloride), sugars (as glucose), lipids (as cholesterol and triglycerides), metabolic waste products (as urea), amino acids, hormones, and vitamins
Plasma thromboplastin antecedent coagulation factor XI
Plasmid a circular bacterial DNA capable of autonomous replication (see also vector)
Plasmin an enzyme that breaks insoluble fibrin strands down into small soluble fragments (fibrin degradation products)
Plasminogen the inactive precursor of plasmin
Platelet thrombocyte, a formed element of the blood involved in primary haemostasis
Platelet plug an accumulation of platelets at the site of injury in a blood vessel, the end product of primary haemostasis
Platelet count the number of platelets in a given volume of blood, an important laboratory measurement in evaluating bleeding disorders; normal count is about 300,000/µl
Platelet dysfunction a bleeding disorder that occurs when platelets do not function properly, causing prolonged bleeding. Platelet dysfunction is one of the most common causes of abnormal bleeding
Plug someth ing which blocks; in haemostasis, a platelet plug is a mass of platelets that stops bleeding from very small blood vessels
Prekallikrein Fletcher factor, precursor of kallikrein
Primary hemostasis control of bleeding through the formation of a platelet plug
Prion Stands for Proteinaceous infections Particles that lack nucleic acides. They are abnormal isoforms of cellular proteins
PrPd Prion protein Cellular; normal cellular prion protein
PrPsc Prion protein Scrapie-like; abnormal prion protein
Proaccelerin factor V
Proconvertin factor VII
Proenzyme the inactive preform of an enzyme; zymogen
Prognosis outlook; anticipated course or result of an illness
Prophylaxis prevention; an intervention done prior to any signs of disease; eg, in the case of hemophilia, the administration of a FVIII product prior to surgery to prevent bleeding
Protein C a protein involved in the regulation of coagulation by degradation of FVIII
Protein S a protein involved in the regulation of coagulation, a cofactor of Protein C
Prothrombin factor II, precursor of thrombin
Prothrombin activation activation of prothrombin (factor II) to thrombin (factor IIa) mainly by FXa/FVa
Prothrombin complex concentrate (PCC) a preparation of plasma proteins that contains factors II, VII, IX, and X
Prothrombin time (PT) blood test that screens for the response of the extrinsic pathway of coagulation
PT prothrombin time
PTT partial thromboplastin time
Purpura purplish-red discoloration due to bleeding into the skin

R

RCo ristocetin cofactor; mimicking platelet aggregation activity of von Willebrand factor measured in a specific test system
Recessive in genetics, a hidden trait; a recessive gene produces its characteristics only when the recessive gene is present in both members of a corresponding pair of chromosomes; of haemophilia A and of haemophilia B on the X-chromosome is recessive and when present in men, who have only one X-chromosome, the disease becomes apparent. Women usually have a normal X-chromosome that will dominate the haemophilia gene – they become carriers
Recombinant produced by genetic engineering
Recombinant DNA DNA that has been produced by combining genetic material from two different sources; a specific gene is inserted into the genetic material (DNA) of another cell
Red Blood Cell (RBC) any of the hemoglobin-containing cells that carry oxygen to the tissues and are responsible for the red blood color of vertebrate blood
Replacement therapy therapy that involves the replacement of clotting factor that the body is lacking RES reticuloendothelial system or monocyte-macrophage system; the system responsible for the clearance of foreign or unphysiological substances in the body
Retroperitoneal behind the peritoneum (the membrane lining the abdominal and pelvic cavities)
rFVIII recombinant factor VIII
rFIX recombinant factor IX
rFVlla activated recombinant factor VII
Ristocetin cofactor see RCo

S

SD virus inactivation method in manufacturing that is effective on enveloped viruses only

Secondary hemostasis final control of bleeding through the formation of a fibrin blood clot
Sephadex an ion exchange resin used for purifying proteins and drugs on chromatography colours
Sex-linked in genetics, a characteristic that is carried on the sex-differentiating X or Y-chromosome
Sindbis virus a model virus enveloped RNA-virus
Size exclusion chromatography chromatography that separates molecules based mainly on their relative sizes
Solvent a material that serves to dissolve another agent
Solvent/detergent (SD) a mixture of a solvent and detergent used to inactivate lipid-enveloped viruses
Spiking when referring to blood products, the addition of a microorganism such as a virus to a product at the beginning of processing, to see how well the processing eliminates the virus
Spontaneous bleeding heavy bleeding that occurs without injury
Stuart-Prower factor factor X
Supernatant the liquid that remains above when an insoluble material settles or after centrifugation; the liquid portion of plasma that remains when cryoprecipitate is separated from plasma
Synovitis inflammation of the joint membrane (synovial membrane).

T

Thrombin (factor IIa) enzyme that converts fibrinogen to fibrin
Thrombin time (TT) blood test for the response of the thrombin-fibrinogen reaction
Thrombocyte blood platelet
Thrombocytopathy impairment of platelet function
Thrombocytopenia reduced number of platelets in the blood
Thrombosis the formation of an occluding platelet plug inside a vessel
Thrombus Blood clot
Thrombogenic clot-forming tendency
Tissue factor (factor III) tissue thromboplastin
Tissue plasminogen activator (tPA) an enzyme released from endothelial cells that activates plasminogen to plasmin, thereby dissolving fibrin
Tissue thromboplastin (factor III) tissue factor
TNBP (tri-n-butyl phosphate) an organic solvent
tPA See tissue plasminogen activator
Transfect to deliver DNA to a cell line
TT See thrombin time

U

Ultrafiltration/diafiltration a purification process, such as that employed in the production of Monoclate® P to remove previously added alcohol and bring the solution to a desired salt concentration

V

Vasopressin a polypeptide hormone that is secreted together with oxytocin by the posterior lobe of the pituitary gland. Also obtained synthetically, Vasopressin increases blood pressure and exerts an antidiuretic effect
vCJD variant Creutzfeldt-Jakob disease
Vector a DNA molecule (plasmid) onto which a gene from another species can be incorporated, normally used to transport the gene to a host cell (transfection)
Venipuncture inserting a cannula into a vein
Virus a microorganism capable of transmitting disease; viruses are smaller than bacteria and need host cells for their reproduction
Virus inactivation In production of plasma products, production step able to inactivate potentially contaminating viruses
Virus reduction in production of plasma products, the ability of the product's processing to reduce the amount of potentially contaminating viruses present in the raw material
Vitamin K a fat-soluble vitamin necessary for the synthesis of factors II, VII, IX, and X in the liver; normally produced in the bowel by the bowel's normal bacteria
Vitamin K-dependent factor a clotting factor that requires vitamin K for its synthesis; factors II, VII, IX, and X
VSV modul virus for enveloped RNA viruses
VWD von Willebrand disease
VWF von Willebrand factor
VWF:Ag vWF antigen
VWF:RCo von Willebrand factor ristocetin cofactor activity
VWF:CB von Willebrand factor collagen binding activity

W

White blood cell (WBC) blood cells that are colorless, lack hemoglobin, contain a nucleus, and include the lymphocytes, monocytes, neutrophils, eosinophils and basophils

Y

Y chromosome a sex chromosome present only in males; normal males have one X chromosome and one Y chromosome

Z

Zymogen when referring to clotting factors, the inactive or non-activated form of the factor or enzyme, proenzyme


Last Updated: 5/19/2010 1:16 PM
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