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Glossary of Terms Specific to Coagulation


Acquired hemophilia hemophilia acquired due to the development of inhibitory antibodies, usually toward factor VIII (FVIII), causing a low plasma FVIII activity level in a person with previously normal blood clotting. These autoantibodies -- which most often occur in the presence of autoimmune disease, pregnancy, and lymphoid malignancy -- are more prevalent in people older than 60 years.
Afibrinogenemia absence of fibrinogen; congenital or acquired anti-hemophilic factor (AHF)
Albumin, human serum a protein that comprises a large proportion of the total protein in normal human plasma. It serves several functions including the maintenance of the blood volume within blood vessels. Albumin is added to some biological products to help stabilize their potency in manufacturing, shipping, and storage.
Antibody (or immunoglobulin) large Y-shaped molecule used by the immune system to identify and neutralize foreign objects like bacteria and viruses. An antibody contains two sites that recognize a target, known as an antigen. Derived from plasma cells, an antibody’s primary function is to fight bacteria, viruses, toxins and other substances foreign to the body.
Anticoagulant a substance that serves to prevent blood from clotting (such as coumarin or heparin)
Antigen any substance capable of inducing a specific immune response and reacting with the products of that response, i.e., with specific antibody, specifically-sensitized T lymphocytes, or both
Antihemophilic (antihaemophilic) factor A factor VIII (FVIII)
Antihemophilic (antihaemophilic) factor B factor IX (FIX), also known as Christmas factor
Arthropathy degenerative joint disease
AT III antithrombin III, also referred to as AT
Autosomal inheritance inheritance through autosomes, rather than through sex chromosomes. The pattern may be dominant or recessive, with males and females typically affected with equal frequency.


B19 parvovirus a non-enveloped human virus; the cause of "fifth disease," a mild disease in children. May cause arthritis, spontaneous abortions, and serious congenital disease of infants born to affected women. Can affect the bone marrow, leading to anemia in immunocompromised patients.
Beriate® P a high-purity plasma-derived factor VIII concentrate
Berinin® P a high-purity plasma-derived factor IX concentrate
Beriplast® P Combi-Set freeze-dried human fibrin sealant for topical application
Beriplex® P/N A highly purified prothrombin complex concentrate (PCC)
Bethesda unit (BU) quantification of inhibiting antibodies in the blood. 1BU decreases the activity by 50%
BHK Baby Hamster Kidney cell line
Bleeding time a global hemostatic test measured as the time a standard skin cut takes to stop bleeding
Bovine viral diarrhea virus (BVDV) a model virus for Hepatitis C used to test the viral reduction capacity of the manufacturing process of plasma-derived products BT
BT See Bleeding time
BU See Bethesda unit
BVDV See Bovine viral diarrhea virus


Ca++ calcium ion
Carrier genetic term, an individual with one allele for a recessive trait. The trait is usually not evident in the carrier, but is likely to be passed to half the carrier's children. Women may be carriers of hemophilia A or B.
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
Christmas disease older name for hemophilia B, the inherited factor IX deficiency
Christmas factor factor IX
Chromosome structure in the nucleus containing a linear thread of DNA which transmits genetic information. A normal human cell has 46 chromosomes, 2 x 22 autosomes and 2 x 1 sex-chromosomes (X or Y chromosome).
CJD Creutzfeldt-Jakob disease
Clotting disorder abnormality in the process of blood clotting
Clotting factors plasma proteins involved in the clotting of blood; coagulation factors
Coagulation clotting of blood
Coagulation disorder see Clotting disorder
Coagulation factor see Clotting factors
Cofactor when referring to blood clotting, a protein such as factor V or VIII required for the normal clotting process to proceed, but not involved as an enzyme in the coagulation cascade. Also known as a catalyst or accelerator.
Collagen connective tissue protein present in skin, tendons, bones, cartilage, and so on
Complementary DNA (cDNA) DNA synthesized by reverse transcriptase using messenger RNA (mRNA) as a template
Congenital present at birth
Consumption coagulopathy decreased coagulability of the blood, caused by excessive consumption of available clotting factors in blood
Coumarin oral anticoagulant taken to inhibit the production of the vitamin K-dependent factors II, VII, IX, and X. It also inhibits the production of the anticoagulant proteins C and protein S.
Cryoprecipitation 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 von Willebrand factor, factor VIII and fibrinogen


Desmospressin acetate the acetate salt of 1-deamino, 8-D-arginine vasopressin, a synthetic analog of vasopressin, used for mild forms of hemophilia A and von Willebrand disease
Detergent when referring to the preparation of a plasma product, a substance which causes viral inactivation by dissolving the lipid membrane of enveloped viruses
DIC disseminated intravascular coagulation. A serious complication of several diseases, characterized by a disturbance of the balance between pro- and anti-coagulant factors, leading to an abnormal coagulability
DNA deoxyribonucleic acid; the nuclear carrier of genetic information
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, congenital or acquired


Ecchymosis bleeding beneath the skin
Edema/oedema tissue swelling due to accumulation of fluid in body tissues
Elastin a connective tissue protein present in the skin
Endothelial cell the type of cell that lines the inside of blood vessels
Endothelium the layer of endothelial cells that lines the cavities of the heart and blood vessels
Enzyme a protein that catalyzes biochemical reactions without being consumed
Epistaxis nose bleeding
Extrinsic pathway coagulation pathway initiated by injury to tissue (exposure to/release of tissue factor = factor III)


Factor I (FI) fibrinogen
Factor II (FII) prothrombin
Factor III (FIII) tissue factor; tissue thromboplastin
Factor IV (FIV) Ca++
Factor V (FV) proaccelerin
Factor VII (FVII) proconvertin
Factor VIII (FVIII) antihemophilic factor A
Factor IX (FIX) Christmas factor or antihemophilic factor B
Factor X (FX) Stewart-Prower factor
Factor XI (FXI) plasma thromboplastin antecedent
Factor XII (FXII) hageman factor
Factor XIII (FXIII) fibrin stabilizing factor
Fibrin an insoluble protein, the end product of the coagulation reactions which stabilizes a blood clot
Fibrin stabilizing factor factor XIII
Fibrinogen factor I, the precursor of fibrin
Fibrinolysis breakdown of a fibrin clot
Fibrogammin® P factor XIII concentrate manufactured by CSL Behring
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 collection from a donor. Contains all clotting factors; usually not virus inactivated


Gene a segment of DNA that contains the information needed for the biosynthesis of a protein
Glycoprotein a protein with carbohydrate moieties (sugar)


Haemate-P® a highly-active von Willebrand factor/factor VIII concentrate. See also Humate-P®.
Haemocomplettan® P a pasteurized fibrinogen concentrate
Hageman factor factor XII
Half-life the length of time needed for the concentration of a substance or drug to reach half of its original concentration
HAV hepatitis A virus
HbSAg hepatitis B surface antigen
HBV hepatitis B virus
HCV hepatitis C virus
Helixate® FS/NexGen the brand of recombinant antihemophilic factor VIII (human) manufactured by Bayer Corporation and distributed by CSL Behring
Hemarthrosis/haemarthrosis blood in a joint
Hematoma/haematoma bleeding into, or collection of blood in, tissue or muscle
Hematuria/haematuria bleeding into, or blood within, urine
Hemolysis/haemolysis destruction, lysis of red blood cells
Hemophilia/haemophilia an inherited disorder of blood coagulation. Hemophilia is characterized as hemophilia A (factor VIII deficiency) and hemophilia B (Factor IX deficiency); bleeding complications depend on the severity of the disease.
Hemophilia A/haemophilia A X-chromosome-linked, recessive disorder of blood coagulation caused by a deficiency or abnormality of factor VIII
Hemophilia B/haemophilia B X-chromosome-linked, recessive disorder of blood coagulation caused by a deficiency or disorder of factor IX
Hemorrhage/haemorrhage bleeding
Hemorrhagic/haemorrhagic pertaining to, or characterized by, bleeding
Hemostasis/Haemostasis stoppage of bleeding
Hemostatic/haemostatic pertaining to stoppage 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 this virus is 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
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 See high-molecular-weight kininogen
Human immunodeficiency virus HIV, the cause of AIDS (HIV-1 and HIV-2)
Humate®-P a highly active von Willebrand factor/factor VIII concentrate (US/Canada brand name)
Hypofibrinogenemia lower-than-normal blood concentration of fibrinogen; can be congenital or acquired


Idiopathic thrombocytopenic purpura (ITP) purpura associated with a decreased number of blood platelets
Immune tolerance special form of treatment for patients who have developed alloantibodies (inhibitors) against coagulation factors, such as factor VIII, with the goal of making the immune system tolerant toward that coagulation factor
Immunoadsorption removal of inhibitory antibodies, e.g. against factor VIII by immunoadsorbent columns
Immunoglobulin a group of plasma proteins, also known as antibodies, synthesized by plasma cells. These proteins help to protect the body against infections. There are five major classes: IgA, IgD, IgG, IgM, and IgE.
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
Inhibitor an antibody against a coagulation factor that interferes with coagulation factor function and complicates replacement therapy. See also immune tolerance.
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 the plasma (XII, XI, X, IX, VIII, V, II, I)
In vitro observable in a test tube
In vivo occurring in the body
Ion a molecule or atom carrying an electrical charge
Ischemia/ischaemia insufficient blood flow through the tissue leading to lack of oxygen
ITI immune tolerance induction; the process of eliminating inhibitory antibodies against FVIII or FIX therapy
ITT immune tolerance therapy; the process of eliminating inhibitory antibodies against FVIII or FIX therapy
IU International Unit, an internationally agreed upon unit of measuring something, such as activity of a coagulation factor


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


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.


MAb/MAbs monoclonal antibody/monoclonal antibodies
Menorrhagia prolonged and/or heavy menstrual bleeding
Monoclonal identical cells or proteins; cells (clones) derived from a single, genetically-distinct cell, or proteins produced by these cells
Monoclonal antibody a specific antibody obtained from a clonal cell culture
Monoclate-P® a CSL Behring brand of freeze-dried human factor VIII concentrate
Monocyte a mononuclear leukocyte in the blood; monocytes can develop into macrophages in the tissue
Monomer a simple molecule of relatively low molecular weight, which is capable of reacting chemically with other molecules to form a polymer, in which the monomers are linked
Mononine® freeze-dried human factor IX concentrate
Multimer a compound consisting of a number of smaller compounds (monomers)
Murine pertaining to rodents, particularly mice
Mutation a change in the structure of a gene; may lead to disease


Nanogram one-billionth of a gram; l0-9 g
Necrosis death, usually applied to tissue
ng nanogram, one-billionth of a gram
nvCJD new variant Creutzfeldt-Jakob disease, same as vCJD


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


Paresis partial or complete paralysis of muscles
Partial thromboplastin time (PTT) a blood test able to screen factor activities of the intrinsic pathway of coagulation
Parvovirus B19 see B19 parvovirus
Pasteurization a unique method of pathogen inactivation introduced for factor VIII products by CSL Behring in the late 1970s. A very effective method to inactivate enveloped and non-enveloped viruses, but gentle enough to assure high product quality.
PCC see prothrombin complex concentrate
Petechia tiny red dot in the skin due to pinpoint bleeding, caused by platelet abnormalities or vasculitis
Pharmacokinetics a pharmacological discipline that describes changes in the concentration of drugs in the organism over time. In particular, questions related to adsorption, distribution, metabolism, and elimination of a given drug are addressed.
Phospholipid any of various phosphorous-containing lipids that are composed mainly of fatty acids, a phosphate group, and a simple organic molecule.
PK pharmacokinetic
PL phospholipid
Plasma the fluid part of blood, consisting of water, salts (electrolytes), and proteins including albumin, immunoglobulins, and coagulation factors
Plasma derived referring to biologic products made from human plasma
Plasmin an enzyme which breaks insoluble fibrin strands down into small soluble fragments (fibrin degradation products)
Plasminogen the inactive precursor of plasmin
Platelet count the number of platelets in a given volume of blood, an important laboratory measurement in evaluating bleeding disorders; normal count is about 150.000 – 450.000/µl
Platelet plug an accumulation of platelets at the site of injury in a blood vessel, the end product of primary hemostasis
Platelet thrombocyte a formed element of the blood, involved in primary hemostasis
Prekallikrein Fletcher factor, precursor of kallikrein
Primary haemostasis control of bleeding through the formation of a primary platelet plug
Prion proteinaceous infectious particles; an abnormal form of protein. Some prions can be infectious; however, not in the same way as viruses or bacteria. Prions are linked to a number of diseases called transmissible spongiform encephalopathies (TSEs), but have never been described as transmissible via plasma derived products.
PrPc Prion protein Cellular; normal cellular prion protein
PrPsc Prion protein Scrapie-like; abnormal prion protein
Proaccelerin factor V
Proconvertin factor VII
Proenzyme the inactive precursor of an enzyme; zymogen
Prophylaxis prevention of, or protective treatment for, disease
Protein C a protein involved in the regulation of coagulation by inactivation of factor V and factor VIII
Protein S 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 (prothrombinase complex)
Prothrombin complex concentrate (PCC) a preparation of plasma proteins that contains factors II, VII, IX, and X
Prothrombin time (PT) blood test which screens for the response of the extrinsic pathway of coagulation
PT see prothrombin time
PTT partial thromboplastin time
Purpura purplish-red discoloration due to bleeding into the skin


RCo ristocetin cofactor; a measure for the platelet aggregation activity of von Willebrand factor
Recessive pertaining to or describing a gene, the effect of which is masked or hidden if there is a dominant gene at the same locus. If both genes are recessive and produce the same trait, the trait is expressed in the individual.
Recombinant DNA DNA that has been produced by combining genetic material
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


SD standard deviation
S/D solvent detergent. Virus inactivation method effective on enveloped viruses only.
Secondary hemostasis final control of bleeding through the formation of a fibrin blood clot
Sex-linked a characteristic that is carried on the sex chromosome
Sindbis virus a model virus enveloped RNA-virus
Solvent a material which serves to dissolve another agent
Solvent/detergent SD, a mixture of a solvent and a detergent, used to inactivate lipid-enveloped viruses
Stuart-Prower factor factor X
Synovitis inflammation of the joint membrane (synovial membrane)


Thrombin factor IIa, enzyme which converts fibrinogen to fibrin
Thrombin time (TT) blood test for the response of the thrombin-fibrinogen reaction to form fibrin
Thrombocyte blood platelet
Thrombocytopathy impairment of platelet function
Thrombocytopenia reduced number of platelets in the blood
Thrombogenic clot-forming tendency
Thrombus blood clot
Tissue factor factor III; tissue thromboplastin
Tissue plasminogen activator (tPA) enzyme released from endothelial cells which activates plasminogen to plasmin
Tissue thromboplastin factor III; tissue factor
TNBP tri-n-butyl phosphate; an organic solvent
tPA tissue plasminogen activator
Transglutaminase a family of enzymes that catalyze the formation of a covalent bond between a free amine group and the gamma-carboxamid group of protein- or peptide-bound glutamine
TT thrombin time


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


vCJD variant Creutzfeldt-Jakob disease
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 by the liver; normally produced in the bowel by the bowel's normal bacteria
Vitamin K-dependent factor a clotting factor which requires vitamin K for its synthesis, as well as factors II, VII, IX, and X, protein C and protein S
von Willebrand disease (VWD) the partial or complete deficiency of von Willebrand factor or, in some cases, the presence of abnormal von Willebrand factor that is not completely functional. Symptoms include mucosal bleeding such as epistaxis (nose bleeds) or menorrhagia (heavy menstrual bleeding), easy bruising, or bleeding after surgery or trauma. In severe cases, gastro-intestinal and joint bleeds may be present.
von Willebrand factor a protein in plasma, platelets, and endothelial cells that serves to anchor platelets to a damaged blood vessel, and to carry factor VIII in the plasma to protect it from degradation.
VSV Vesicular stomatitis virus, modul virus for enveloped RNA viruses
VWD von Willebrand disease
VWF von Willebrand factor
VWF:Ag von Willebrand factor antigen
VWF:CB von Willebrand factor collagen binding activity
VWF:RCo von Willebrand factor ristocetin cofactor activity


Y chromosome a sex chromosome present only in males


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

Last Updated: 6/1/2012 7:08 AM
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