The word ‘immune’ refers to the resistance from infections and ‘deficiency’ refers to lack of protection or failure of the immune system. In this slide types of immunodeficiency, their characteristics, signs and symptoms, treatment and prevention are to be discussed.

1. IMMUNODIFICIENCY:
PLAN:
 Introduction
 Types of immunodeficiency
disease.
 Signs and symptoms
 Diagnosis
 Treatment and Prevention
INTRODUCTION:
Immunology is the study of immune systems which
protect our body from infections and diseases. The
two major types of immune system are: 1.Innate
immunity and 2. Acquired Immunity.
What is IMMUNODEFICIENCY?
The word ‘immune’ refers to the resistance from
infections and ‘deficiency’ refers to lack of
protection or failure of the immune system. These
disorders prevent our body from fighting infections
and diseases. The immunodeficiency may be the

2. result of inherited genetic defect or may be
developmental defects or even may be due to
external agent.
TYPES:
 PRIMARY IMMUNODIFICIENCY
 SECONDARY IMMUNEDIFICIENCY
PRIMARY IMMUNODIFICIENCY:
 The immunodeficiency resulting from inherited
genetic defects and developmental defects in the
immune system. The defect is present in birth.
 To date over 150 different types of primary
immune disorders has been identified.
 Primary immune disorders are mainly caused
due to the mutation in a specific gene.
 About 60% of people with primary
immunodeficiency are male.
 Problems with B cells and their production
causes: Common Variable Immunodeficiency,
Selective Immunoglobulin deficiency, X-linked
agammaglobulinema.

3.  Problems with T cells are chronic
mucocutaneous candidiasis, X linked lymph
proliferative syndrome.

4.  Problems with combined B and T cells are
Severe Combined Immunodeficiency, Ataxia
syndrome, Hyperimmunoglobulinemia E
syndrome.
 Problems with Phagocytosis are Chronic
granulomatous disease, Leukocyte adhesion
disease.
 Deficiency in complement proteins C1
inhibitory deficiency.
SECONDARY IMMUNODEFICIENCY
 Secondary immunodeficiency, also known as
acquired immunodeficiency, is the loss of
immune
function that results from exposure to an
external agent, often an infection.
 The disorders can result from drugs, radiation
exposure and chronic disorders such as cancer
and diabetes.
 Human Immunodeficiency Virus infection
results in Acquired Immunodeficiency
Syndrome, the most common severe acquired
immunodeficiency disorder.

5.  Many types of cancer can cause an
immunodeficiency disorder. For example, any
cancer that affects the bone marrow (such as
leukemia and lymphoma) can prevent the bone
marrow from producing normal white blood
cells (B cells and T cells), which are part of the
immune system.

6. SIGNS AND SYMPTOMS:
 People with an immunodeficiency disorder tend
to have one infection after another.
 Usually, respiratory infections (such as sinus and
lung infections) develop first and recur often.
 Most people eventually develop severe bacterial
infections that persist, recur, or lead to
complications. For example, sore throats and
head colds may progress to pneumonia.
 Infections of the mouth, eyes, and digestive tract
are common.
 Thrush, a fungal infection of the mouth, may be
an early sign of an immunodeficiency disorder.
 People may have chronic gum disease
(gingivitis) and frequent ear and skin infections.
 Bacterial infections (for example, with
staphylococci) may cause pus-filled sores to
form (pyoderma).
 People with certain immunodeficiency disorders
may have many large, noticeable warts (caused
by viruses).
 Many people have fevers and chills and lose
their appetite and/or weight.

7.  Abdominal pain may develop, possibly because
the liver or spleen is enlarged.
 Infants or young children may have chronic
diarrhea and may not grow and develop as
expected (called failure to thrive).
Immunodeficiency may be more severe if
symptoms develop in early childhood than if
they develop later.
 Other symptoms vary depending on the severity
and duration of the infections.
 Primary immunodeficiencies may occur as part
of a syndrome with other symptoms. These other
symptoms are often more easily recognized than
those of the immunodeficiency.
DIAGNOISIS:
Blood Test: Blood tests can determine if you have
normal levels of infection-fighting proteins
(immunoglobulin) in your blood and measure the
levels of blood cells and immune system cells.
Abnormal numbers of certain cells can indicate an
immune system defect.
Blood tests also can determine if your immune
system is responding properly and producing

8. antibodies — proteins that identify and kill foreign
invaders such as bacteria or viruses.
Prenatal Testing: Parents who've had a child with a
primary immunodeficiency disorder may want to be
tested for certain immunodeficiency disorders during
future pregnancies. Samples of the amniotic fluid,
blood or cells from the tissue that will become the
placenta (chorion) are tested for abnormalities. In
some cases, DNA testing is done to test for a genetic
defect. Test results make it possible to prepare for
treatment soon after birth, if necessary.
TREATMENT:
STEMCELL TRANSPLANTATION:
Stem cell transplantation offers a permanent cure for
several forms of life-threatening immunodeficiency.
Normal stem cells are transferred to the person with
immunodeficiency, giving him or her a normally
functioning immune system. Stem cells can be
harvested through bone marrow, or they can be
obtained from the placenta at birth (cord blood
banking).

9. The stem cell donor — usually a parent or other
close relative — must have body tissues that are a
close biological match to those of the person with
primary immunodeficiency. Even with a good
match, however, stem cell transplants don't always
work.
The treatment often requires that functioning
immune cells be destroyed using chemotherapy or
radiation before the transplants, leaving the
transplant recipient temporarily even more
vulnerable to infection.
MANAGING INFECTIONS:
Treating infections: Infections require rapid and
aggressive treatment with antibiotics. Infections that
don't respond may require hospitalization and
intravenous (IV) antibiotics.
Preventing Infections: Some people need long-term
antibiotics to prevent respiratory infections and
associated permanent damage to the lungs and ears.
Children with primary immunodeficiency may not
be able to have vaccines containing live viruses,
such as oral polio and measles-mumps-rubella.