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Glossary
1. Activated partial thromboplastin time (aPTT)– This test is used to see if
a person has a clotting or bleeding problem like hemophilia. It measures the
time it takes for a clot to form after certain substances have been added to
the blood sample. The normal time is between 16 to 40 seconds.
2. Adapter – A device used to connect two things together.
3. Allergic reaction – A reaction of the immune system to a foreign
substance; sometimes patients can have an allergic reaction to clotting factor
replacement medicines.
4. Anaphylaxis – A serious, sometimes life-threatening allergic reaction to a
foreign substance.
5. Anemia – A lower number of red blood cells in the body than normal.
6. Antibody – Substances made by the immune system in response to antigens
or foreign substances. Antibodies are unique and protect the body against a
specific antigen. In people with hemophilia B, the body may develop
antibodies, called inhibitors, that stop the clotting factor replacement
medicine from stopping a bleed.
7. Bethesda assay – A test used to find out if the body had developed
inhibitors to clotting factor replacement treatment.
8. Bleeding time – Used as part of screening for hemophilia. A test that
measures the time it takes for blood to stop flowing from a small wound.
9. Blood clotting factors – Substances in the blood that are needed to make
the blood clot.
10. Carrier – A person who has one gene that gives information for a specific
disease, and one normal gene. Because of the normal gene, this person may
not show any signs or symptoms of the disease, but they may be able to
pass along the disease to their children.
11. Cellulitis – An infection of the skin and the tissue just under the skin.
12. Chromosome – Long pieces of hereditary information found in the center of
each cell. Humans have 23 pairs of chromosomes (46 total). Half come from
the mother and half come from the father.
13. Coagulation – The process by which blood cells clump together to form a
clot.
14. Continuous infusion – A controlled way to deliver a liquid drug into the
body through a vein over a prolonged period of time. The safety and efficacy
of BeneFIX® [Coagulation Factor IX (Recombinant)] by continuous infusion
have not been established.
15. Deficient – Less than what is considered normal; for example, a person with
hemophilia is deficient in clotting factor activity.
16. Diluent –A liquid used to dissolve a solid substance to make it a liquid ready
for infusion.
17. Diluent syringe – The syringe containing the diluent or liquid used to
dissolve the medicine.
18. Gene – The basic unit of DNA that makes up chromosomes. Genes contain a
person’s hereditary information.
19. Hemophilia – A blood disorder in which a person’s blood does not clot
properly and they bleed longer than normal.
20. Hemophilia Treatment Center – a treatment facility that provides a range
of comprehensive services for patients with bleeding disorders and their
families.
21. Hypersensitivity – The body’s reaction to a foreign substance.
22. Hypoxia – A lack of oxygen.
23. Immune tolerance induction – A process in which factor concentrate is
given regularly over a period of time so the body gets trained to recognize
the treatment without reacting to it.
24. Infusions – Injecting a liquid (other than blood) into the body through a
vein.
25. Infusion port – A silicone case that is placed under the skin that has a
catheter (tube) threaded into a vein. The port allows long-term access to a
vein.
26. Inhibitor – Something that stops or blocks another substance. In hemophilia
B, inhibitors are antibodies that stop clotting factor replacement medicine
from helping to form a blood clot.
27. Luer end – A type of tip on a syringe that allows for quick and firm
attachment of the needle.
28. Mutations – A change to hereditary information that may be passed on to
children; not all mutations are harmful, but some may cause diseases.
29. Nanofiltration – a process of filtering a liquid or a drug to remove large
molecules like viruses or proteins.
30. Nephrotic syndrome – The result of any number of diseases that damage
the kidneys. Nephrotic syndrome causes a large amount of protein in the
urine, low protein levels in the blood, high cholesterol, and swelling.
31. Phlebitis – Pain and tenderness of a vein.
32. Prothrombin time (PT) – This test is used to see if a person has a clotting
or bleeding problem, like hemophilia. This test measures how long it takes for
a person’s blood to clot after certain substances are added.
33. Recombinant – When genetic material is combined from different sources.
34. Recombinant DNA – Refers to DNA that has been altered by joining genetic
material from different sources. A recombinant DNA molecule can enter a
cell, multiply, and be made into a protein. BeneFIX® [Coagulation Factor IX
(Recombinant)] is a purified protein produced by recombinant DNA
technology.
35. Reconstitution – The process of adding a liquid, called a diluent, to a
powdered medicine to make a solution.
36. Renal infarct – The death of tissue in the kidney caused by a blocked artery
in the kidney.
37. Surgical prophylaxis – Administering clotting factor replacement around
the time of an operation, or surgery. Also called peri-operative management.
38. Synovium – A layer of tissue that lines the inside of joint cavities. It
secretes a fluid that lubricates the joint.
39. Tourniquet – A device that is used to apply pressure over a vein to make it
easier to inject medicine.
40. Vial – A small bottle of medicine.