Primary immune deficiency diseases:
    An overview

Riding the subway … dusting the furniture … going to school or work … shaking someone's hand. These are everyday, normal activities most of us take for granted. For people with primary immune deficiency disease (PI), however, these simple tasks are often accompanied by infection, pain, or at the very least, anxiety about the situation at hand.

PI diseases result from defects in various components of the immune system that generally are inherited. They comprise a diverse group of illnesses that affect thousands of children, adolescents and adults. These primary disorders differ from secondary or acquired immune deficiency disorders in which an external factor, such as a virus or a drug, is responsible for the immune deficiency.

PIs usually result from a deficiency of antibody production. One of the building blocks of immunity, antibodies-also known as immunoglobulin-are protein substances produced in tissues and circulating in blood, which help destroy or inactivate bacteria, viruses and toxins. PIs are a group of diseases in which the immune system's ability to produce specific antibodies to fight off infection is greatly reduced or absent. These problems lead to an increased susceptibility to infection. Treatment includes immunoglobulin replacement, generally administered in the form of immune globulin intravenous (IGIV). For patients diagnosed with these types of primary immune deficiencies, IGIV is a life-saving and life-long therapy.

Due to the availability of IGIV, the face of PI is now changing. Fifty years ago, most people with PI went undiagnosed, and having this disease essentially amounted to a death sentence.

Even when diagnosed, people with PI usually did not survive into adulthood, or suffered permanent impairment, such as loss of lung function, loss of hearing or persistent abnormalities of digestive functions. Today, PI is no longer a pediatric disease. IGIV has given many people with PI not only hope, but a quality of life into adulthood that previously did not exist.

Despite the availability of IGIV therapy today, many people with PI remain undiagnosed and therefore untreated. The average time for diagnosis is over nine years from the onset of symptoms. PI still affects thousands of children, adolescents and adults who do not even know they have the disease. They often suffer from recurrent infections and may experience serious and sometimes permanent disability because they remain undiagnosed for too long.

There is a critical need for education of physicians and patients to help raise awareness and recognition of the signs and symptoms of PI. With early diagnosis and treatment, people with PI can expect to lead a nearly normal life and lifespan similar to those in the general population.

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Idiopathic Thrombocytopenic Purpura:      An overview

IGIV is also used when the immune system malfunctions and attacks the body's own tissue and cells. It then affects the immune system to prevent damage to the body. This immune-modulating effect is seen when IGIV is used to treat patients with Idiopathic Thrombocytopenic Purpura (ITP).

In patients with ITP, the body destroys platelets by producing anti-platelet antibodies that attach to the body's own platelets. The immune system eliminates the platelets "thinking" that they may be intruding pathogens. This results in a decrease in platelet count (thrombocytopenia), despite the body's attempt to step up production of platelets. The result is an abnormally low platelet count that can lead to occasional life-threatening bleeding episodes. IGIV is used to protect against that platelet destruction response.

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