IMMUNODEFICIENCY DISEASE

General Information

DEFINITION--Defects in the body's immune system. A healthy immune system protects the body against germs (bacteria, viruses and fungi), cancer (partial protection) and any foreign material that enters the body. When the system fails, the body becomes susceptible to infection and cancer. Similar, but entirely different disorders are caused by acquired immune deficiency syndrome (AIDS). Also different is the immunosuppression some people develop due to potent drugs used to treat several disorders.

BODY PARTS INVOLVED--Immune system (blood, bone marrow, lymph tissue, liver, spleen and thymus gland).

SEX OR AGE MOST AFFECTED--Both sexes; all ages.

SIGNS & SYMPTOMS--

Recurrent, severe infections and illnesses. The most common include:

• Ear or respiratory infections, such as otitis media and pneumonia.
• Yeast infections, especially candidiasis.
• Cancer, especially leukemia and lymphoma.
• Bleeding disorders.
• Eczema.
• Meningitis or encephalitis.

CAUSES

• Congenital defects that involve an incomplete or absent immune system.
• Surgical removal of the spleen before age 2.
• Use of immunosuppressive drugs.
• Radiation treatment.
• Some cancers, such as Hodgkin's disease.
• Hypogammaglobulinemia (See Glossary).
• Viral infections.

RISK INCREASES WITH--Family history of immunodeficiency disease.

HOW TO PREVENT--No preventive measures known. If you have a family history of immunodeficiency disease, seek genetic counseling before starting a family. Prenatal amniotic fluid culture analysis may identify some of these disorders.

What To Expect

DIAGNOSTIC MEASURES--

• Your own observation of symptoms, especially repeated infections in children.
• Medical history and physical exam by a doctor.
• Laboratory blood studies of antibodies, microscopic examination of blood and tissue cells and skin tests.
• Chest x-rays of the thymus gland.
• Radioactive studies of immune function.

APPROPRIATE HEALTH CARE

• Doctor's treatment.
• Surgery to transplant bone marrow or the thymus gland (occasionally).
• Hospitalization for treatment of serious infection.

POSSIBLE COMPLICATIONS

• Uncontrolled bacterial, viral or fungal infections that don't respond to treatment.
• Cancer.
• Infectious arthritis.

PROBABLE OUTCOME--Severe forms of immunodeficiency are usually fatal. Minor forms can be treated successfully.

How To Treat

GENERAL MEASURES--

• Treatment will depend on the complexity of the immune deficiency. Basically, goals are to maintain optimal health, prevent emotional problems and manage infections.
• Avoid exposure to persons with contagious illnesses.
• Don't take any type of vaccine without medical advice.
• See Resources for Additional Information.

MEDICATION--Your doctor may prescribe:

• Antibiotics to fight infections.
• Injections of antibodies.
• Transfusions of blood components.
• Injections of gamma globulin (sometimes).

ACTIVITY--Bed rest is usually necessary during acute illnesses. Otherwise, there are no restrictions on activity.

DIET--No special diet.

Call Your Doctor If

• You have symptoms of immunodeficiency disease.
• After diagnosis, you have signs of infection, such as: chills; fever; muscle aches; headache; dizziness; and cough with thick, discolored or blood-streaked sputum.