disorders of immunity

1. DISORDERS OF IMMUNE SYSTEM
IMMUNODEFIC
IENCY
PRIMARY
Defects in
Innate
Immunity
Defects in
Adaptive
Immunity
SECONDARY
Defects in
Leukocyte
Function
Defects in
Complement
System
Defect in B cell
Defect in T cell
Leukocyte adhesion deficiency
(LAD) I, II, III
X-Linked
Agammaglobulinemia
DiGeorge Syndrome

2. DEFECT IN LEUKOCYTE FUNCTION
DISORDER OF
PHAGOCYTE
ADHESION AND
CHEMOTAXIS
Leukocyte
adhesion
deficiency
(LAD) I
Leukocyte
adhesion
deficiency
(LAD) II
Leukocyte
adhesion
deficiency
(LAD) III
DISORDER OF
RESPIRATORY BURST
PATHWAY
DEFECT IN STRUCTURE
AND FUNCTION OF
LYSOSOMAL ENZYME
CHEDIAK
HIGASHI
SYNDROME
Chronic
Granulomatous
Disease

3. LAD
I
Autosomal recessive
Absence of all 3 beta integrin
Reduced CD18
Delayed umblical cord
separation
Periodontitis
LAD
II
Impaired E – selectin
Normal CD 18 and absent CD 15
Defective FUCOSE metabolism
Common among BOMBAY
BLOOD group
SLC35C1 gene sequencing
LAD
III
Autosomal recessive
Defcet in KINDLIN 3
Normal CD 18 and CD 15
FERMT3 gene sequencing

4. DISORDER OF
RESPIRATORY BURST
PATHWAY
Nitroblue tetrazolium
test
Semiquantitative assay
Dihydro rhodamine 123
- flowcytometry
X linked disease
NADPH oxidase
deficiency
Mutation in CYBB
gene
Chronic
granulom
atous
disease

5. CHEDIAK
HIGASHI
SYNDROME
CHS1 gene
mutation
LYST defect
Occulocutaneous
albinism
Recurrent
infection and
easy bruising
Defect in primary
granule formation
and platelet ADP
release