Immune Deficiencies

Common variable immunodeficiency is characterized by low levels of most or all immunoglobulin classes, and recurrent bacterial infections such as recurrent ear, sinus, bronchial or lung infections. Severe, recurrent infections can cause permanent damage to the bronchial tubes. Also, patients with common variable immunodeficiency often have gastrointestinal complaints.

X-linked agammaglobulinemia is found only in men because it’s an inherited disorder of the X chromosome. Men with X-linked agammaglobulinemia can’t produce antibodies. Their immature B-cells fail to become mature, infection-fighting B-lymphocytes. These patients are prone to develop serious bacterial or viral infections. And they’re susceptible to a few viruses that cause serious, life-threatening illness, such as hepatitis.

Severe combined immunodeficiency (SCID) results from a severe defect in T- and B-lymphocyte development. This disorder makes patients highly susceptible to serious and complicated infections. Infants with SCID have an unusual number of severe-even life-threatening-bacterial, viral, fungal or protozoal infections. Pulmonary infections (such as pneumonia), fungal infections and persistent diarrhea are common in patients with SCID.

Immune thrombocytopenic purpura (ITP) is a disorder of the blood. The main symptom is bleeding. "Purpura" refers to a characteristic purple discoloration of the skin and mucous membranes in these patients. Chronic ITP can be mild or severe.

Guillain-Barré syndrome(GBS) is a disorder in which the body's immune system attacks part of the peripheral nervous system.

Treatment

Igs with high levels of specific antibodies are called hyperimmune immunoglobulins. They include products to treat tetanus, rabies, hepatitis B and to prevent hemolytic disease of the newborn. Human anti-D immunoglobulin prevents women who are Rh-negative from acquiring antibodies to an Rh-positive fetus. Giving anti-D to the mother protects future infants from hemolytic disease of the newborn, a condition that develops in an Rh-positive fetus when anti-Rh antibodies produced by the Rh-negative mother have passed through the placenta and attack red blood cells in fetal circulation, which is caused by the incompatibility between the mother’s blood type and her child’s.

Hyperimmune immunoglobulins are administered following solid organ transplant surgery to prevent the development of cytomegalovirus infection, or CMV. CMV is a potentially life-threatening infection with a peak onset of one to four months after transplant. Transplant patients are susceptible to CMV because they are taking immunosuppressant therapies to prevent organ rejection.

Human normal immunoglobulin for IV administration is indicated for the treatment of patients with primary immunodeficiencies, such as common variable immunodeficiency, X-linked agammaglobulinemia, severe combined immunodeficiency, ITP and combined immunodeficiency. Human normal immunoglobulin for subcutaneous administration is indicated for the treatment of primary immune deficiency.