Lack of one or more components of the immune system results in immunodeficiency
disorders. These can be inherited, acquired through infection or
other illness, or produced as an inadvertent side effect of certain
drug treatments.
People
with advanced cancer may experience immune deficiencies as a result
of the disease process or from extensive anticancer therapy. Transient
immune deficiencies can develop in the wake of common viral infections,
including influenza, infectious mononucleosis, and measles. Immune
responsiveness can also be depressed by blood transfusions, surgery
malnutrition, and stress.
Some
children are born with defects in their immune systems. Those with
flaws in the B cell components are unable to produce antibodies
(immunoglobulins). These conditions, known as agammaglobulinemias
or hypogammaglobulinemias, leave the children vulnerable to infectious
organisms; such disorders can be combated with injections of immunoglobulins.
Other
children, whose thymus is either missing or small and abnormal,
lack T cells. The resultant disorders have been treated with thymic
transplants.
Very
rarely, infants are born lacking all the major immune defenses;
this is known as severe
combined immunodeficiency disease (SCID). Some children with
SCID have lived for years in germ-free rooms and "bubbles." A few
SCID patients have been successfully treated with transplants of
bone marrow (Bone Marrow Transplants).
The
devastating immunodeficiency disorder known as the acquired
immunodeficiency syndrome (AIDS) was first recognized in 1981.
Caused by a virus (the human immunodeficiency virus, or HIV)
that destroys T4 cells and that is harbored in macrophages as well
as T4 cells, AIDS is characterized by a variety of unusual infections
and otherwise rare cancers. The AIDS virus also damages tissue of
the brain and spinal cord, producing progressive dementia.
AIDS infections are known as "opportunistic" because they are produced
by commonplace organisms that do not trouble people whose immune
systems are healthy, but which take advantage of the "opportunity"
provided by an immune defense in disarray. The most common infection
is an unusual and life-threatening form of pneumonia caused by a
one-celled organism (a Protozoa)
called Pneumocystis carinii. AIDS patients are also susceptible
to unusual lymphomas and Kaposi's sarcoma, a rare cancer that results
from the abnormal proliferation of endothelial cells in the blood
vessels.
Some
persons infected with the AIDS virus develop a condition known as
AIDS-related complex, or ARC, characterized by fatigue, fever, weight
loss, diarrhea, and swollen lymph glands. Yet other persons who
are infected with the AIDS virus apparently remain well; however,
even though they develop no symptoms, they can transmit the virus
to others.
AIDS
is a contagious disease, spread by intimate sexual contact, by direct
inoculation of the virus into the bloodstream, or from mother to
child during pregnancy. Most of the AIDS cases in the United States
have been found among homosexual and bisexual men with multiple
sex partners, and among intravenous drug abusers. Others have involved
men who received untreated blood products for hemophilia; persons
who received transfusions of inadvertently contaminated blood-primarily
before the AIDS virus was discovered and virtually eliminated from
the nation's blood supply with a screening test; the heterosexual
partners of persons with AIDS; and children born to infected mothers.
There
is presently no cure for AIDS, although the antiviral agent zidovuzine
(AZT) appears to hold the virus in check, at least for a time. Many
other antiretroviral drugs are being tested, as are agents to bolster
the immune system and agents to prevent or treat opportunistic
infections. Research on vaccines to prevent the spread of AIDS
is also under way.