Immunology

Immune Deficiency Disorders
In general, immunodeficiency means that one or more components of the immune system are either absent or functioning abnormally.

People with primary immunodeficiency disease (PID) are born with an immune system that doesn’t produce enough antibodies in the blood to fight infection. Primary immunodeficiency disorders are caused by inherent, often genetic, defects in the cells of the immune system.

Primary immunodeficiency causes children and adults to have infections that recur frequently and are usually hard to cure. Warning signs of primary immunodeficiency include recurrent ear, nose, or chest infections; deep skin or organ abscesses; failure of an infant to gain weight or grow normally; frequent need for intravenous (IV) antibiotics to clear infections; a family history of primary immune deficiency; and other warning signs.

The World Health Organisation currently recognizes more than 100 different PIDs.

Unlike primary immunodeficiency, which is built in to the immune system, secondary immunodeficiency is acquired and may be the result of other serious disorders such as cancer or following a bone marrow transplantation.

Since the discovery of congenital immunodeficiency disease, a large number of other immunodeficiency disorders have been described. Patients with immunodeficiency disorders are susceptible to recurrent infections caused by bacteria and opportunistic infections caused by common yeasts and viruses.

CSL Behring UK Ltd provides the following products to treat immune deficiency disorders. Please click on the individual product name to access its Patient Information Leaflet:

• Privigen^®
• Vivaglobin^®

For further information on immune deficiency disorders, you can visit The Primary Immunodeficiency Association’s website at www.pia.org.uk.

Hereditary Angioedema
Hereditary angioedema (HAE) is a rare, genetic condition that can cause considerable swelling in various body tissues.

Depending on the severity of the disease, some people will have many attacks a month, while others could go months or even years without an attack.

Swelling can occur in the hands, feet, arms, legs, face, throat and genitals. Patients can also experience unexplained abdominal pain.

The treatment of HAE aims to halt the progression of any swelling as quickly as possible and is particulary important with swelling of the throat which can obstruct the airway and be life-threatening.

CSL Behring UK Ltd provides the following product to treat HAE. Please click on the product name to access its Patient Information Leaflet:

• Berinert^®

For further information on HAE, you can visit The Primary Immunodeficiency Association’s website at www.pia.org.uk.