Primary Immune deficiency, secondary immune deficiency, clinical warning, diagnosis

1. Immune Deficiency: Diagnostic
Approach
Prof Ariyanto Harsono MD PhD SpA(K)

2. Introduction
Immune disorders are oftentimes categorized as either
congenital or acquired. When baby born with a
disorder, it’s called a congenital or primary disorder.
Acquired disorders are called secondary disorders.
Secondary disorders are more common than primary
The immune system is made up of several organs
including spleen, tonsils, bone marrow, and lymph
nodes. These organs make and release lymphocytes,
which are white blood cells classified as B cells and T
cells. B and T cells fight invaders called antigens..
Prof Ariyanto Harsono MD PhD SpA(K)
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3. Prof Ariyanto Harsono MD PhD SpA(K)
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4. Function of Major Immune
Component
Prof Ariyanto Harsono MD PhD SpA(K)
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5. Immune Deficiency
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6. Primary disorders can be controlled and treated,
but not prevented. Secondary disorders can
be prevented in a number of ways. For
example, it is possible to avoid AIDS by not
having unprotected sex with someone who
carries the virus.
Prof Ariyanto Harsono MD PhD SpA(K)
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8. Diagnosis steps
Prof Ariyanto Harsono MD PhD SpA(K)
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9. Recognizing the Signs of an
Immunodeficiency Disorder
Each disorder has unique symptoms. The first
indicator that something is wrong with your
immune system is becoming repeatedly and
chronically sick, even with minor illnesses,
including pinkeye, sinus infections, or diarrhea. If
these problems don’t respond to treatment or
don’t completely get better over time, doctor
might test for a disorder. Recurrent pneumonia
and yeast infections could also suggest you have a
disorder.
Prof Ariyanto Harsono MD PhD SpA(K)
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13. Recurrent oral thrush
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19. Advance Laboratory Examination
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21. Alternative Algorythm
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24. Specific Condition
Hyper IgE Syndrome
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25. Primary Immunodeficiency Disease
According to the leading experts in immunology,
when part of the immune system is either absent
or not functioning properly, it can result in an
immune deficiency disease. When the cause of
this deficiency is hereditary or genetic, it is called
a primary immunodeficiency disease (PIDD).
Researchers have identified more than 150
different kinds of PIDD.
Prof Ariyanto Harsono MD PhD SpA(K)
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26. Severe Combined Immunodeficiency
Severe Combined Immunodeficiency (SCID) is a
rare, inherited condition resulting in a weak
immune system that is unable to fight off even
mild infections. It is considered to be the most
serious primary immunodeficiency disease
(PIDD).
Prof Ariyanto Harsono MD PhD SpA(K)
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27. Prof DR.dr. Ariyanto Harsono SpAK

28. X-Linked Agammaglobulinemia
X-Linked Agammaglobulinemia (XLA) is an
inherited immunodeficiency in which the body
is unable to produce the antibodies needed to
defend against bacteria and viruses.
Prof Ariyanto Harsono MD PhD SpA(K)
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29. Selective IgA Deficiency
Selective IgA Deficiency is the most common
primary immunodeficiency disease (PIDD).
People with this disorder have absent levels of
a blood protein called immunoglobulin A
(IgA). IgA protects against infections of the
mucous membranes lining the mouth, airways
and digestive tract.
Prof Ariyanto Harsono MD PhD SpA(K)
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30. DiGeorge Syndrome
DiGeorge Syndrome (DGS) is a primary
immunodeficiency disease(PIDD) associated
with susceptibility to infections due to poor T
cell production and function. DGS is caused by
abnormal cell and tissue development during
fetal growth. In addition to possible immune
system problems, this abnormal development
can result in altered facial characteristics,
abnormal gland development or defects in
organs such as the heart.
Prof Ariyanto Harsono MD PhD SpA(K)
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31. DiGeorge Syndrome…..
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32. Wiskott Aldrich Syndrome
Wiskott-Aldrich syndrome is an X-linked
recessive immunodeficiency disorder
characterized in one third of patients by the
triad of recurrent bacterial sinopulmonary
infections, eczema (atopic like dermatitis), and
a bleeding diathesis caused by
thrombocytopenia and platelet dysfunction.
Prof Ariyanto Harsono MD PhD SpA(K)
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33. Wiskott Aldrich Syndrome…
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34. Ataxia-telangiectasia
Ataxia-telangiectasia (A-T) is an autosomal
recessive, complex, multisystem disorder
characterized by progressive neurologic
impairment, cerebellar ataxia, variable
immunodeficiency with susceptibility to
sinopulmonary infections, impaired organ
maturation, x-ray hypersensitivity, ocular and
cutaneous telangiectasia (see image below), and a
predisposition to malignancy.
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37. Secondary Immunodeficiency
Syndrome
Also called Acquired immunodeficiency may be a
complication of diseases such as HIV infection and
malnutrition. Many cancers may also cause
immunodeficiency.
People who have had their spleen removed have an
acquired immunodeficiency, and are at higher risk
for infection by certain bacteria that the spleen
would normally help fight. Patients with diabetes
are also at higher risk for certain infections.
Prof Ariyanto Harsono MD PhD SpA(K)
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38. Clinical Features of secondary immune
deficiency
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Syndromes
Failure to Thrive
Bacterial infection
Viral Infection
Opportunistic infection
Chronic diarrhea
Blood abnormality
Skin lesions
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56. Thank you
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