IMMUNODEFICIENCY DISEASE

A.) Disease of phagocytic cells

1. (CGD )chronic granuomatous disease

2.(LAD) leukocyte adhesion deficiency

3.Cheadiak higashi synddrome

4. G6PDH

5.Myeloperoxidase deficiency

6.Job's syndrome

DEFECT OH HUMORAL IMMUNITY:

1. Bruton X aggamaglobulinemia

2. selective IG A deficiency

3. X-linked hyper IG M syndrome

4. Common variable immunodeficiency

5. transient hypogammaglobulinemia of infancy

DEFECT of T-Lymphocyte

1. Selective t-cell deficiency:

a. digeorge syndrome

b. MHC class I deficiency

c. MHC class II deficiency

2.Combined partial B & T cel deficiency

a. wiskott-adrich syndome

b.ataxia teleangiectasia

3. Complete functional B& T cell deficiency

a. SCID( Severe combined Immunodeficiency)

DEFICIENCY OF COMPLEMENT OR ITS REGULATION

1.classical pathway -c1q,c1r,c1s,c4,c2
2.alternative pathway factor B and properdin
3.Both pathway -a. C3  b. C5,C6,C7,C8(MAC)
4.deficiency in complement regulatory protein -C1-INH(hereditary angioedema)

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1. Chronic granulomatous disease
Molecular defect function : deficiency of NADPH Oxidase,failure to generate oxygen radicals.
Symptoms: recurent infection with catalase positive bacteria and fungi.

important diagnosis for CGD: Nitro-Bue tetrazolium test (NBT) negative=normal positive=CGD

2. Leukocyte adhesion deficiency

-Absence of CD18 common beta chain of the leukocyte integrin

sympotoms: recurrent infecr fail to form pus omphalitis