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Immunoglobulins

Introduction

Immunoglobulins (antibodies) are proteins that circulate in the blood. They provide protection from diseases by binding to foreign proteins (for instance the proteins that are present on infectious viruses or bacteria), inhibiting activity and forming large complexes which are rapidly cleared from the circulation. There are 2 broad types of immunoglobulin preparations, "normal" and "specific".

Normal intravenous immunoglobulin (IVIG) preparations are used in the treatment of patients who have no, or very low levels of, antibody production. This may be due to a genetic disorder, to disease, or to treatment such as chemotherapy.  They are also used in the treatment of certain auto-immune conditions.

Specific immunoglobulins are preparations that contain a high concentration of antibodies to particular viruses or bacteria. If these products are given soon after exposure to the virus or bacterium concerned (e.g. soon after being bitten by a rabid animal), they will help to fight infection. This is particularly important in cases where the affected person has not previously been vaccinated against the virus or bacterium. In such cases it takes several days before the patient's own immune system produces effective antibodies.

Normal immunoglobulins

Normal intravenous immunoglobulin (IVIG) preparations are used in the treatment of patients who have no, or very low levels of, antibody production. This may be due to a genetic disorder, to disease, or to treatment such as chemotherapy.

Primary Immunodeficiency (PID)

This results from the failure of the immune system to produce sufficient antibodies to fight infection. The most common types of PID are:

  • Common Variable Immunodeficiency (CVID) which affects males and females equally, with low levels of all immunoglobulin classes.
  • IgG subclass immunodeficiency where one or more of the 4 main IgG subclasses are absent or deficient.
  • X-linked Antibody Deficiency (X-LA) which is also known as Bruton's Disease. This is an inherited condition in which the body fails to produce the cells that make antibodies. While this condition affects males only, females, however, act as carriers, and may pass the condition to male children.

Secondary Immunodeficiency (SID)

SID is a shortage or complete lack of antibodies which results from the effects of another illness.  An example of a SID is Chronic Lymphocytic Leukaemia (CLL).  It is a disease that prevents B-cells from working properly. B-cells are the cells which produce antibodies, so patients with CLL often lack sufficient antibodies to fight infection. For patients with low levels of antibodies and recurrent infections, regular doses of IVIG are needed to maintain antibody levels high enough to protect against infection.

As for PID, treatment with IVIG boosts antibody levels, allowing patients to fight infection efficiently. Regular doses of IVIG are needed as long as the condition persists.

Idiopathic Thrombocytopenic Purpura (ITP)

This is a disease which results in a shortage of platelets. It is caused by the patient's own antibodies attacking his or her platelets. Low levels of platelets can result in bleeding problems. In children, ITP often appears after an infection and usually goes away within a few weeks without treatment. In adults, ITP is less likely to go away spontaneously and becomes chronic. Spontaneous remission is less likely with adults and, if platelet counts fall very low or there are bleeding tendencies, treatment may be needed. There are many possible treatments but IVIG may be used because it blocks the normal breakdown of platelets by the spleen.

Link to the ITP Support website
www.itpsupport.org.uk

Kawasaki Disease

This is thought to follow an infectious disease and mainly appears in children, resulting in inflammation of the blood vessels and other tissues, such as heart muscle. About 25% of affected children develop heart problems during the later stages of the illness. A high dose of IVIG given early in the course of the disease in conjunction with aspirin can prevent coronary artery damage. The mode of action of IVIG in this condition is not understood, but it is extremely effective.

Guillain-Barré Syndrome (GBS)

GBS is a short-term (acute) inflammation of the nerves that control the arms and legs. Although the exact cause of GBS is not fully understood, it often follows an infectious disease, e.g. of the gastro-intestinal tract. It is known that the symptoms are caused by the patient's own antibodies attacking the nerves to the limbs. The damage caused by the antibodies accounts for the tingling and short term paralysis symptomatic of GBS. IVIG can be used in the treatment of this condition, the wide variety of antibodies infused helping to cancel the effect of the nerve-damaging antibodies.

Our immunoglobulins

BPL has three presentations of Normal Immunoglobulins. Two presentations are for intravenous administration (directly into a vein). They are available as 2.5 g, 5 g, and 10 g doses, already in solution for immediate administration.

The third presentation of Normal Immunoglobulin is for injection under the skin (subcutaneous administration).  It is a sterile liquid supplied as 750 mg and 1500 mg doses.  This presentation is indicated for primary immunodeficiency, secondary hypogammaglobulinaemia in patients with CLL or myeloma with recurrent infections and children with congenital AIDS who have repeated bacterial infections.

For further documentation visit the Patient/Carer resource centre

Specific immunoglobulins

Specific immunoglobulins are preparations that contain a high concentration of antibodies to particular viruses or bacteria.  These products are usually injected into a muscle.  If they are given soon after exposure to the virus or bacterium concerned (e.g. soon after being bitten by a rabid animal), they will help to fight infection. This is particularly important in cases, where the affected person has not previously been vaccinated against the virus or bacterium. In such persons, it takes several days before the patient's own immune system produces effective antibodies; specific immunoglobulins can help provide protection (usually lasting a few weeks).

Patient information leaflets