A

Acquired hemophilia

A form of hemophilia in which the body starts producing antibodies that fight its own blood clotting proteins.

Activated partial thromboplastin time (aPTT)

Measures clotting time of the blood to determine the condition of the intrinsic and common pathways of the blood-clotting process.

Activated platelet

Tiny cells in the blood that stick to an injured vessel and to each other to form a plug.

Administer

In terms of hemophilia, it means to apply to the body through infusion.

Albumin

A water-soluble protein found in the blood.

Analgesics

Painkiller medications.

Anamnestic response

An increase in inhibitor level that can occur quickly the second time the body is exposed to factor replacement therapy.

Antibodies

Cells that the immune system produces to fight and destroy viruses and bacteria that can cause infection.

Antifibrinolytic

A type of substance that decreases the breakdown of fibrin, keeping blood clots intact. Antifibrinolytic therapy helps maintain the blood clots that the body forms to stop bleeding.

Aseptic

Free of disease-spreading microorganisms.

Autosomal recessive disorder

An inherited disorder having to do with an autosome, which is a chromosome that is not a sex (gender) chromosome. Both parents must have the disorder to pass it on to their child.

B

Bacteria

One-celled pathogens that reproduce quickly and can cause illness.

Bethesda inhibitor assay

A test performed in the lab and used to measure the strength or titer of the inhibitor. Inhibitors are reported in Bethesda Units (BU).

Bleed

A collection of blood in an area, such as a muscle or joint.

Bolus

A large dose of medicine that is given at the beginning of treatment to raise blood-level concentrations to a therapeutic level.

Bypassing products

A product that skips the need for Factor VIII or Factor IX in treating a bleed.

C

Capillaries

A network of very tiny blood vessels throughout the body that helps various substances circulate between blood and tissue cells.

Cartilage

A tough connective tissue found in various parts of the body, such as the joints, outer ear, and larynx.

Central nervous system

The part of the nervous system consisting of the brain and spinal cord, which coordinate all impulses that circulate through the body.

Chromosome

A threadlike structure, found in every cell, that contains all of a person's genetic information.

Classic hemophilia

Another name for hemophilia A, the most common type of hemophilia.

Clinical trials

Carefully coordinated studies to determine the effectiveness and safety of medicines and treatment strategies.

Clot

The plug your body forms at the place where you've been injured to stop the bleeding.

Clotting

The process of forming into a clot or clots.

Clotting assay

A blood test given to determine what specific bleeding disorder a person has, based on a prolonged PT or PTT previously measured.

Clotting (coagulation) factors

Substances in the blood that work together with platelets to form a fibrin clot in an injured blood vessel and stop bleeding. Clotting factors are named using Roman numerals (Factor I [one] to Factor XIII [thirteen]).

Coagulation

Also known as blood clotting. It is the process of turning some of the elements in the blood into a clot. Coagulation is the result of a sequence of actions and reactions that involve several different proteins in the blood.

Co-infection

To be infected with more than one illness at the same time.

Comprehensive care

Treatment at a doctor's office where patients will see a variety of doctors about different aspects of their health and body.

D

Deficiency

A lack or shortage of something.

Desensitize

To treat a person so their immune system does not react to a certain foreign substance by developing antibodies.

Diluent

A substance that changes the consistency of something else. For example, water dilutes iced tea powder to make tea that people drink.

DNA

Deoxyribonucleic acid. DNA contains all of a person's genetic information and is found in every cell in that person's body.

E

Electrolytes

Various minerals in the bloodstream that regulate the electric charge of the cells.

Eradicate

To eliminate or get rid of.

External bleeding

Bleeding on the outside of the body, such as from cuts and scrapes.

F

Factor

A protein in the blood that helps form blood clots.

Factor IX (nine) deficiency

Absence or shortage of Factor IX (nine), one of the 13 factors that help form blood clots. Also called hemophilia B.

Factor replacement

Replacing deficient or absent factor protein in the body with donated or synthetic factor of the same type.

Factor VII (seven) deficiency

A rare bleeding disorder in which the blood has low levels of Factor VII. Factor VII is important in the blood-clotting process because it binds to blood cells near the location of an injury and to activated platelets, setting the clotting process in motion.

Factor VIII (eight) deficiency

Absence or shortage of Factor VIII, one of the factors that help form blood clots. Also called hemophilia A.

Fibrin

A substance in the blood that combines with blood cells and platelets to form a stable clot at the site of bleeding.

Fibrin clot

Fibrin that works together with a group of clotting factors and blood cells to make a patch (a stable clot) and stop bleeding.

G

Gene

A section of DNA. Each gene on a strand of DNA contains certain genetic traits that have been passed on from parents to child.

Genetics

The science of heredity and how genes transfer to offspring.

H

Hemarthrosis

When blood accumulates in a joint, causing extreme swelling. Repeated bleeding of this type over time can cause a permanent condition called hemophilic arthropathy.

Hematologist

A doctor who is an expert in blood diseases, such as hemophilia, acquired Factor VIII inhibitors, and other clotting disorders.

Hemophilia

A clotting disorder that occurs mostly in males. The disorder makes bleeding hard to control.

Hemophilia A

A bleeding disorder caused by lack of Factor VIII. It is sometimes called classic or standard hemophilia.

Hemophilia B

A bleeding disorder caused by lack of Factor IX.

Hemophilia Treatment Center (HTC)

A medical facility that specializes in the comprehensive treatment of hemophilia and other clotting disorders.

Hemophilia with inhibitors

A bleeding disorder where the body fights against the factor used to treat hemophilia.

Hemophilic arthropathy

Chronic joint disease in hemophilic individuals.

Hemorrhage

Excessive and sometimes uncontrollable bleeding.

Hemostasis

The stopping of bleeding through the blood-clotting process.

High-responding

Type of inhibitor that responds quickly with high levels of antibodies to factor replacement therapy.

Histidine

An essential amino acid which is present in many proteins.

Home therapy program or Home treatment (therapy)

A program in which the patient or a caregiver administers factor treatment, when needed, in the home setting, thereby avoiding having to travel to a hospital or HTC.

I

Immune system

A mechanism within the body used to protect itself from harmful substances.

Immune tolerance therapy (ITT or ITI)

Treatment in which high doses of Factors VIII or IX are used to lower inhibitor levels in patients with hemophilia A or B.

Infusion

The injection of medicine directly into a vein using a needle and syringe.

Inhibit

To hold back, make difficult, or prohibit.

Inhibitor

A substance produced by the body that attacks factor so it cannot work as well. For example, a Factor VIII inhibitor would keep Factor VIII from working with other factors and platelets to stop bleeding.

Insurance caps

The amount of money an insurance company pays to cover an illness or procedure. A lifetime cap is the amount of money an insurance company will spend during the lifetime of the policy holder. Yearly caps refer to a maximum amount that can be spent per year.

Internal bleeding

Bleeding inside the body, in tissues or organs.

Intraoperative

Something that occurs during a surgical operation.

Invasive procedure

Most often referred to as surgery, because the doctor enters the body through an incision to repair damage.

J

There are currently no glossary items for the letter "J."

K

There are currently no glossary items for the letter "K."

L

Low-responding

Type of inhibitor that has a slow, weak response to factor replacement therapy.

M

Mutation

A spontaneous change in the structure of a gene or chromosome, which leads to a new trait not found in parents or any family.

N

Neutralize

To counteract the effect of something so that it isn't capable of producing any change.

O

Obligate carrier

Someone who has a disease-causing gene but does not actually have the disease because he or she has one normal gene.

On-demand therapy

Treatment that responds to a need when it occurs, instead of before it occurs.

Orthopedic surgery

Surgical treatment for diseases, disorders, and injuries involving bones, joints, and tendons. These surgical treatments are performed by orthopedic surgeons.

P

Pathogen

An organism, such as a virus, prion, or bacterium, that causes disease. An emerging pathogen is a pathogen that has recently or not yet been discovered.

Plasma

The fluid part of the blood that contains clotting factors and other proteins.

Plasmapheresis

A process that removes harmful components from the blood.

Platelets

Tiny cells in the blood that stick to an injured vessel and to each other to form a plug.

Postoperative

Something that happens after a surgical operation.

Prion

A type of pathogen that is known to cause diseases that attack brain tissue.

Prolonged

Something that has taken a longer time in duration than normal.

Prophylaxis

Factor replacement therapy that is given on a regular basis to keep a bleeding episode from starting.

Prothrombin

A substance in the blood essential to forming stable clots.

Prothrombin time (PT)

Measures clotting time of the blood to determine the condition of the extrinsic and common pathways of the blood-clotting process.

Q

There are currently no glossary items for the letter "Q."

R

Recombinant

Genetically engineered DNA. Made without human blood or plasma.

Reconstitution

Adding liquid to prepare a medicine.

Red blood cells

Cells in the blood that transport oxygen and carbon dioxide.

Rehabilitation

To restore to good health.

Resolve

To cause reduction in something, such as swelling or bleeding.

R.I.C.E.

Stands for rest, ice, compression, and elevation. It's a type of therapy that can relieve pain and discomfort during a bleed, particularly in a joint.

S

Spontaneous

Something that happens on its own, for what seems like no reason. Spontaneous bleeding doesn't appear to have a specific cause.

Surgeon

A doctor specializing in surgery, which is a class of operations that involves operating through an open incision in the skin to get to damaged parts within the body.

T

Thrombin

Also known as Factor IIa (2a). It's a protein that binds together other proteins and activated platelets to start forming a clot. Thrombin is created when activated Factor X (Factor Xa) comes in contact with prothrombin.

Thrombosis

An event that takes place when veins or arteries become blocked by platelets that are working to "plug" an injury, preventing blood from flowing normally through the body, and possibly causing a stroke.

Titer

Concentration or strength of a substance; measures the strength of an inhibitor in a person's blood.

Tolerability

Capability of being put up with or endured.

Tolerate

The ability to endure doses of a medicine

Transient

Something that stays in place temporarily and then disappears on its own, such as an inhibitor, which is an antibody to coagulation factors that are brought into the body.

U

There are currently no glossary items for the letter "U."

V

Virus

A pathogen that enters healthy cells and reproduces into large numbers, which then spread through the bloodstream and air passages.

W

White blood cells

Cells in the blood that help protect the body from infection and disease.

X

X-linked

Having to do with gene transmission along the X chromosome in DNA.

Y

There are currently no glossary items for the letter "Y."

Z

There are currently no glossary items for the letter "Z."