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Immunotherapy Product Line
Areas of therapy
Immunodeficiencies
Immunodeficiencies may occur for a variety of reasons: some of them, the primary immunodeficiencies (PID), are genetically determined and are associated with serious bacterial and viral infections. Others occur either as a consequence of severe underlying haematologic diseases like chronic leukaemia or myeloma or they result from immunosuppression e.g. in bone marrow transplantation (secondary immunodeficiencies, SID).
Primary Immunodeficiencies
Primary immunodeficiency syndromes are a group of genetic disorders in which the body is unable to produce a sufficient amount of antibodies or sufficiently functional antibodies. Children and adults with PID have an increased risk of getting recurrent bacterial and viral infections. Infections typically attack the upper respiratory tract (sinusitis, bronchitis, pneumonia) but can also affect the gastrointestinal tract (gastroenteritis). They can be severe and lead to substantial morbidity. Responses to antibacterial therapy are often poor. At present, most primary immune deficiencies are not curable, but intravenous and subcutaneous immunoglobulins (IVIG, SCIG) have been shown to decrease the total number of severe infections and the duration of hospitalisation.
Secondary Immunodeficiencies
Viral and bacterial infections are a serious cause of morbidity and mortality in immunocompromised patients, e.g. as a result of an underlying malignancy. Some haematological malignancies like myeloma or chronic lymphatic leukaemia (CLL) lead to a reduced production of natural antibodies (immunoglobulins). These diseases are associated with recurrent infections. Intravenous immunoglobulin (IVIG), given in intervals of 3-4 weeks, decreases the total number of severe infections and the number of days in hospital.
Patients undergoing bone marrow transplant may have an increased incidence of infections due to a transient immunodeficiency after transplantation and the effects of immunosuppressive drugs. Administration of sufficient doses of IVIG reduces the risk of getting severe infections.
Immunomodulation
Within the last 20 years there has been a substantial increase in understanding of the immune system, autoimmune diseases and the immunomodulating effects of IVIG therapy. The mode of action of IVIG is complex, as it involves Fc receptor-mediated effects, anti-inflammatory effects, and effects on B- as well as on T-cells.
Idiopathic thrombocytopenic purpura (ITP) / Autoimmune thrombocytopenic purpura (AITP)
ITP is an immune-mediated disease caused by auto-antibodies that induce platelet destruction. It is characterised by marked thrombocytopenia with a drastically shortened platelet life span and a normal or increased platelet production. ITP occurs at all ages, with children mainly displaying the acute form. People with a severe autoimmune thrombocytopenic purpura are at risk of life-threatening bleeding. They require a treatment rapidly increasing the platelet count to avoid severe haemorrhage. IVIG is usually given 2g/kg BW in divided doses over 2-5 days. Several controlled trials have shown that IVIG is a rapid and predictable therapeutic agent for increasing the platelet count.
Guillain-Barré-Syndrome (GBS)
GBS, also called acute inflammatory demyelinating polyneuropathy (AIDP), is an inflammatory disorder of the peripheral nerves. GBS is characterised by a rapidly ascending weakness and, often, paralysis of extremities and breathing muscles. 2g/kg BW IVIG given in divided doses is therapeutically equivalent to plasma exchange in shortening the course of GBS.
Kawasaki-Syndrome
Kawasaki-Syndrome is a disease exclusively affecting young children. About 80% of patients are younger than 4 years. The aetiology of Kawasaki syndrome remains unknown. It may be caused by an infectious agent leading to an immune-mediated syndrome. The diagnosis of Kawasaki syndrome is based on various criteria e.g. fever (generally high spiking), conjunctival injection, and changes of the mucosa of the oropharynx. Approximately 20% of untreated patients with Kawasaki syndrome develop cardiovascular sequelae, including aneurysms. IVIG (2g/kg BW) is recommended in combination with aspirin.
On www.immune-complex.ch you will find information on the pathomechanisms encountered in autoimmune diseases and the mechanism of action of immunoglobulin G therapy.
(Please note that clicking on this link will take you outside of the Octapharma web site. Please mind that this is an external web page and Octapharma declines all responsibility for its contents.)
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