GLOSSARY OF TERMS

Active Bleeding

Actively bleeding includes and is not limited to nose bleed, menstruation, rectal, passing urine and so on and patients should contact their primary health care professional.

Acute ITP

ITP is called newly diagnosed within 3 months of initial diagnosis. In children, this usually follows a viral infection and generally recovery to a normal platelet count will occur with or without steroid treatment. In adults, the majority will not recover to a normal platelet count and treatment is needed if the platelet count is less than 20-30 x10^9/l or if bleeding complications occur.

Antibody

an-ti-bahd-ee

A protein produced by the immune system which binds to an antigen and causes destruction. EG. Vaccination with the tetanus vaccine causes the body to produce antibodies directed against the tetanus bacteria. If the tetanus bacteria enter the body through a wound those antibodies are ready to bind to the bacteria and cause their destruction.

Antigen

an-ti-juhn

Any substance within the body which the immune system recognises as foreign. These can be a virus, bacteria or germ and the immune system responds by producing antibodies to neutralise the antigen.

Antinuclear Antibody Test (ANA)

an-ti-nyOO-kliuh An-ti-bod-ee

The ANA, or Antinuclear Antibody Test, identifies the presence of antinuclear antibodies (ANA) in the blood. ANA is a group of antibodies produced by the patient’s immune system when it fails to distinguish between ‘self’ and ‘non-self’. These autoantibodies may attack the body’s own cells, causing signs and symptoms such as tissue and organ inflammation, joint and muscle pain, and fatigue. The presence of ANA can be a marker of an autoimmune process and is associated with several autoimmune disorders.

Asplenic

Without a spleen, typically meaning a person who has had their spleen removed.

Asymptomatic

When patients have an illness or condition, however, are not displaying any symptoms.

Autoimmune Disease

awt-ohi-myOOn

Autoimmune diseases occur when a person’s immune system produces an antibody against its own cells. This results in inflammation and damage. There are many different autoimmune diseases, including lupus, rheumatoid arthritis and ITP. These diseases can be localised to a single organ or tissue, or generalised (systemic), affecting many body organs and tissues.

Biopsy

bIE-op-see

Removal of tissue from the body for the purpose on making a diagnosis. While the amount of tissue removed varies, in many cases only a tiny sample is required.

Bone Marrow

Bone marrow is found within the bones and is the place where blood cells (red cells, white cells and platelets) are made.

Bone Marrow Biopsy

A bone marrow biopsy is usually performed from the back of the iliac bone (pelvis). Usually, some liquid marrow is drawn out by a syringe (aspirate), and then a core of bone is taken (trephine) so that the marrow can be examined under the microscope by a haematologist. In suspected ITP, the reason for the biopsy is to make sure that there is no other cause (diagnosis) for low platelets. In most people with ITP a bone marrow biopsy is not needed.

Capillaries

Minute blood vessels located close to the surface of the skin.

Chronic ITP

Chronic ITP is diagnosed when patients have ITP continuing for more than 12 months from diagnosis.

Ciclosporin

Ciclosporin, also spelled cyclosporine and cyclosporin, is a calcineurin inhibitor, used as an immunosuppressant medication. The mechanism of its action is the suppression of both mediated immune reaction and production of antibodies mediated by T cells.

Clinical Trial

Clinical trials are research investigations in which patients volunteer to test new treatments, interventions or tests as a means to prevent, detect, treat or manage various diseases or medical conditions. Some investigations look at how people respond to a new intervention and what side effects might occur while others are dose finding investigations to identify the optimal dose of a particular treatment or drug.

Coagulation

koh-a-gyu-lAY-shuhn

Coagulation is the process by which blood changes from a liquid to a gel, forming a blood clot. Coagulation is designed to blocks holes in blood vessels.

Complement System

The complement system (or complement cascade), is a part of the immune system that enhances (complements) the ability of antibodies and phagocytic cells to clear microbes and damaged cells from an organism, promote inflammation, and attack the pathogen’s cell membrane.

Contraindication

Something (such as a symptom or condition) that makes a particular treatment or procedure inadvisable.

Corticosteroid

kawt-i-koh-stIUH-roid

1. Biochemistry – any steroid hormone produced by the adrenal cortex that affects carbohydrate, protein, and electrolyte metabolism, gonad function, and immune response.

2. Pharmacology – any similar synthetic substance, used in treating inflammatory and allergic diseases (including prednisolone, dexamethasone and hydrocortisone)

FBC

Full Blood Count. A sample of blood is examined to measure the size and quantity of red cells, white cells and platelets in one millilitre of blood.

Half Life

The time taken for the drug to reduce its original value. Half-life is used to estimate how long it takes for the drug to be removed from the body.

H Pylori

Helicobacter pylori (H. pylori) is a type of bacteria. These germs can enter your body and live in your digestive tract. After many years, they can cause sores, called ulcers, in the lining of your stomach or the upper part of your small intestine. It has been reported that H Pylori eradication (with antibiotic treatment) can cure some patients with ITP. However, success seems to be much higher in some countries (eg. Japan) than in Australia, USA or Europe.

Idiopathic

id-iuh-pAth-ik

This means that the cause of a condition is unknown. Many conditions which used to be idiopathic (including ITP) now have a known cause. ITP used to be short for idiopathic thrombocytopenia purpura but now stands for immune thrombocytopenia.

Idiopathic Thrombocytopenia Purpura

id-iuh-pAth-ik THROM-bo-si-toe-PE-ne-ah pUHR-pyu-ruh

Shortened to ITP and is now formally known as Immune Thrombocytopenia.

Immune Thrombocytopenia

THROM-bo-si-toe-PE-ne-ah

Immune thrombocytopenia (THROM-bo-si-toe-PE-ne-ah), or ITP, is a bleeding disorder. In ITP, the blood doesn’t clot as it should. This is due to a low number of blood cell fragments called platelets (PLATE-lets) or thrombocytes (THROM-bo-sites).

Immunoglobulin

The protein from which antibodies are made – found in the blood in tissue fluids. Immunoglobulins are produced in the body by cells of the immune system, known as B-lymphocytes. It is also manufactured from human donors and is administered intravenously (IVIg).

Inherited Platelet Disorder

A platelet disorder which is inherited (present from birth)/runs in families. May be incorrectly diagnosed as ITP if no previous blood tests have been performed. Genetic testing can be performed to establish the correct diagnosis in most cases.

International Consensus Report

A report compiled and reviewed by international experts to promote the best approach to diagnosing and treating a condition.

ITP Warrior

A term used to describe a person or patient with ITP.

ITP

Formerly idiopathic thrombocytopenia purpura and was shortened to ITP. It is now formally known as Immune Thrombocytopenia.

IVIg

Intravenous immunoglobulin (IVIg) is a solution of human IgG antibodies with a broad spectrum of antibody activity. IVIg is prepared from blood donations collected from several thousand blood donors and contains IgG antibodies found in the normal population.

LDH

LDH is blood test which indicates increased cell turnover. It is normal in ITP and if raised can indicate an alternative diagnosis such as myelodysplasia or lymphoma OR may indicate the red blood cells are being destroyed by an immune process as well as platelets. This combined platelet/red cell destruction is called Evans syndrome.

Petechiae

pe-TEEK-ee-ay

A small red or purple spot caused by bleeding into the skin.

Peptibody

Part peptide and part antibody.

Persistent ITP

ITP is called persistent when the diagnosis is longer than 3 months (Acute ITP) but has not reached 12 months (Chronic ITP) since diagnosis.

Phagocytosis

When macrophages engulf and digests foreign substances including anything that does not have the type of proteins specific to healthy body cells on its surface.

Platelets

PLATE-lets

A small disc-shaped cell fragment without a nucleus, found in large numbers in blood and involved in clotting. Made by megakaryocytes in the bone marrow and reduced in people with ITP.

Purpura

pUHR-pyu-ruh

A rash of purple spots on the skin caused by internal bleeding from small blood vessels.

Quality of Life – QoL

Quality of Life – QoL – is an overarching term for the quality of the various domains in life. It is a standard level that consists of the expectations of an individual or society for a good life and medical practitioners take a patient’s quality of life into consideration when discussing and administering treatments.

Refractory ITP

ITP which does not respond to treatment. In such cases, less commonly used treatment or clinical trials may be the best option. It is important to be aware that some people with refractory ITP may have been misdiagnosed, and may, for example, have an inherited platelet disorder.

Rituximab

Also knows as Mabthera (retail name), is a manufactured antibody directed against B lymphocytes. Read more here – About Rituximab

Spleen

The spleen is an organ about the size of an orange which is in the abdomen (belly) under the ribs on the left-hand side. It filters blood and removes cells coated with antibodies. This is usually helpful in fighting infections, but in conditions like ITP the platelets coated with antibodies are removed, causing low platelet counts (thrombocytopenia).

Splenectomy

spli-nEk-tuh-mee

An operation that removes the spleen. In ITP two thirds of patients will see a substantial rise in platelet counts following splenectomy. There are some short term and long term risks, including increased risk of serious infections (septicaemia) and blood clots. The risks and benefits need to be carefully weighed up in discussion with your haematologist and the surgeon performing the operation.

Steroids

See Corticosteroids.

Subcutaneous

Under the skin. It is a type of injection, where drugs are injected into the tissue layer of the skin and muscle. Subcutaneous injection is preferred to intramuscular injection in patients with low platelets or bleeding disorders as the risk of bleeding at the injection site is less.

Thrombocytopenia

THROM-bo-si-toe-PE-ne-ah

Low platelet count. A normal platelet count ranges from 150 to 400. In ITP it is rare to have any bleeding problems with a platelet count over 30. It is important to understand that a normal platelet count is not needed for a normal life and in most cases, the aim of treatment in ITP is to get a safe platelet count of 50 or more.

Thrombopoietin

Thrombopoietin is the naturally occurring hormone that controls platelet production from megakaryocytes. It is produced in the liver at a constant rate. Drugs including eltrombopag, romiplostim and avatrombopag perform the same role as thrombopoietin in boosting platelet production.

TPO-RA

The thrombopoietin receptor agonists (TPO-RAs) are manufactured drugs that perform the same role as naturally occurring thrombopoietin, increasing platelet production. Two TPORAs, romiplostim and eltrombopag, are currently available for patients with chronic ITP, provided certain conditions are met. Further TPORAs are under development, including avatrombopag.