The document focuses on various immunodeficiency diseases, categorizing them into B cell, T cell, phagocytic dysfunction, and complement-mediated disorders. It details specific disorders such as X-linked agammaglobulinemia, common variable immunodeficiency, and severe combined immunodeficiency, highlighting their causes, symptoms, and treatment options. Additionally, it outlines the clinical evaluation of immune cells and other related conditions affecting immunity.

1. Immunology

2. Immunodeficiency
Diseases

3. Introduction
B cell mediated immunity
T cell mediated immunity
Two other ancillary system:
Complement system
Phygocytosis

4. Introduction
B cell mediated immunity
T cell mediated immunity
Two other ancillary system:
Complement system
Phygocytosis

5. Introduction
Immunoglobulin (B cell) immunodeficiency
disorder
Cellular (T cell) deficiency disorder
Phygocytic dysfunction
Complement deficiency states

6. Immunoglobulin (B cell)
immunodeficiency disorder
1) X linked infantile hypogammaglobulinaemia
 X link and on long arm of X chromosomes
 Not observed in Infants
 More frequent in male

7. Immunoglobulin (B cell)
immunodeficiency disorder
1) X linked infantile hypogammaglobulinaemia

8. Immunoglobulin (B cell)
immunodeficiency disorder
1) X linked infantile hypogammaglobulinaemia
 Susceptible to Haemophilus Influenza,
pneumococci, strepto cocci, staphylococci,
meningiococci
 Normal resistance to other virus, fungi and Gram –
ve bacteria
 Susceptible to polio

9. Immunoglobulin (B cell)
immunodeficiency disorder
1) X linked infantile hypogammaglobulinaemia
 Susceptible to Haemophilus Influenza,
pneumococci, strepto cocci, staphylococci,
meningiococci
 Normal resistance to other virus, fungi and Gram –
ve bacteria
 Susceptible to polio

10. Immunoglobulin (B cell)
immunodeficiency disorder
1) X linked infantile hypogammaglobulinaemia
 Susceptible diarrhoea and malabsorption
 Death due to dermatomyositis with neurologic
involvement
 Diagnosis
 Lack of B cell and plasma cell
 IM, IV IgG for life

11. A Case of X-linked
Agammaglobulinemia
Billy DeWitt was a normal, full-term baby at birth. Beginning at
about 10 months of age, Billy suffered from a series of infectious
processes such as sinusitis, otitis media, and pneumonia. All of these
conditions were successfully treated with antibiotics, but within a few
weeks of the resolution of one infection, another would occur. Now at
about four years of age, Billy is examined by a pediatrician who notes
that Billy lacks palatine tonsils, although he does not have a history of
tonsillectomy. Questioning of Billy’s mother reveals that she had two
male relatives who died in infancy from infectious disease.

12. A Case of X-linked
Agammaglobulinemia
The physician orders laboratory tests that reveal that the quantity of
immunoglobulin in Billy’s serum is about one-fi fth of normal, and that
there is a marked defi ciency in the number of circulating B-lymphocytes
in Billy’s blood. Tests to determine the functional state of Billy’s T-
lymphocytes are all normal. Billy is diagnosed as having a genetic
disorder called X-linked Agammaglobulinema.

13. A Case of X-linked
Agammaglobulinemia
He begins a course of monthly intravenous injections of gamma globulin
which he will need to continue for the rest of his life. Billy should no
longer suff er recurrent infections and should develop physically and
mentally as a normal child. Aside from receiving monthly injections of
gamma globulin, he should lead a normal life now that this course of
treatment is in place.

14. A Case of X-linked
Agammaglobulinemia
1. What are the means by which antibody molecules
exert a protective effect?
2. Billy was free of infections for the fi rst few months
of life. Why?
3. Why did Billy lack tonsils?
4. Explain X-linked inheritance, and name other
genetic diseases that are known to be X-linked?

15. Immunoglobulin (B cell)
immunodeficiency disorder
2) Common Variable Immunodeficiency (CVID)
Not genetically transmitted
Affect male and Female equally
Occurs at any age
Depressed level of IgG, other Ig also less
B cell not working properly
Cell mediated immunity impaired

16. Immunoglobulin (B cell)
immunodeficiency disorder
Pathogenetic cause
 B cell do not respond to T cell help
 B cell synthesized but cannot secrete antibodies
 Helper T cell are absent
 Autoantibodies to B cell may be present

17. Immunoglobulin (B cell)
immunodeficiency disorder
Infection
Several autoimmune diseases
Malabsorbtion syndromes
Never treated with steroids
Lymphoid hyperplasia
 Treatment

18. Immunoglobulin (B cell)
immunodeficiency disorder
Selective IgA deficiency
 Common in Immunodeficiency
 B cells are normal, problem in secretion
 Related to sinus and pulmonary infection
 Increase in autoimmune, malignant, allergic,
bacterial infection

19. Immunoglobulin (B cell)
immunodeficiency disorder
 Normal cellular immunity
 Healthy patient
 Not given with γ globulins
 Anaphylactic reactions

20. Immunoglobulin (B cell)
immunodeficiency disorder
Immunoglobulin deficiency with elevated IgM
 IgM and IgD increases, low IgG and IgA
 X linked, acquired disorder
 Both men and women
 Haemolytic anameia, neutropenia,
thrombocytopenia
 Lack of T cells

21. Immunoglobulin (B cell)
immunodeficiency disorder
Immunoglobulin deficiency of IgM or sub
classes of IgG
 Onset of CVID
 Selective deficiency of IgG subclasses
 Bacterial infection with capsulated strain
 Diagnostic
 Treatment

22. Immunoglobulin (B cell)
immunodeficiency disorder
Immunoglobulin deficiency of IgM or sub
classes of IgG
 Onset of CVID
 Selective deficiency of IgG subclasses
 Bacterial infection with capsulated strain
 Diagnostic
 Treatment

23. T cell mediated
immunodeficiency disorder
T cell mediated immunity
 Susceptible to opportunistic infection
 Infection is more likely and fatal

24. T cell mediated
immunodeficiency disorder
Congenital Thymic Hypoplasia
(Di george Syndrome)
 Lack of normal development of Pharangeal pouches
 Caused by Intrauterine accident
 Do not exhibit delayed hypersensitivity
 Commonly in child below 5 years of age
 Treatment

25. T cell mediated
immunodeficiency disorder

26. T cell mediated
immunodeficiency disorder
Severe combined Immunodefieciency (SCID)
 Depletion of B and T cells
 Fatal
 Autosomal recessive trait or X linked
 ADA enzyme
 Purine Nucleoside Phosphorylase enzyme
 Reticular digenesis
 Affect long bones
Nezelof’s Syndrome with B cells

27. T cell mediated
immunodeficiency disorder
Severe combined Immunodefieciency (SCID)
 Depletion of B and T cells
 Fatal
 Autosomal recessive trait or X linked
 ADA enzyme
 PNP enzyme
 Reticular digenesis
 Affect long bones
Nezelof’s Syndrome

28. T cell mediated
immunodeficiency disorder
Severe combined Immunodefieciency (SCID)
 3-6 month
 Chronic pulmonary infection, Diarrhoea
 Diagnosis
 Treatment

29. T cell mediated immunity
Wiskott – Aldrich Syndrome
 X linked Affecting boys
 Eczema, Thrombocytopenia
 Lack isohaemagglutinins, cannot make
antibodies to polysaccariedes
 Antibody to protiens are evident
 IgG normal, IgE, IgA high, IgM low
 Treatment

30. T cell mediated immunity
Wiskott – Aldrich Syndrome
 X linked Affecting boys
 Eczema, Thrombocytopenia
 Lack isohaemagglutinins, cannot make
antibodies to polysaccariedes
 Antibody to protiens are evident
 IgG normal, IgE, IgA high, IgM low
 Treatment

31. T cell mediated immunity
Immuno defieceiency with ataxia telangiectasia
Ataxia telangiectasia
 Neurological disorder

32. Phygocytic Dysfunction
Diseases
Phygocytosis

33. Phygocytic Dysfunction
Diseases
Enzyme deficiency for killing bacteria
Susceptibility to infection
More susceptible to bacterial and fungal

34. Phygocytic Dysfunction
Diseases
1) Chronic granulomatous diseases
 X linked disorder
 Observed in first two years
 Susceptible to unusual infections
 Lymphodenitis, hepatosplenomegaly, pneumonia

35. Phygocytic Dysfunction
Diseases
1) Chronic granulomatous diseases


36. Phygocytic Dysfunction
Diseases
2) Specific enzyme deficiency
 Glucose 6 phosphate dehydrogenase
 Leucocyte myeloperoxidase
 Alkaline phosphatase

37. Phygocytic Dysfunction
Diseases
3) Chediak Higashi Syndrome
 Autosomal recessive disorder
 Bacterial infection, hepatosplenomegaly, partial
albinism, CNS abnormality
 Delayed killing
 Respiratory burst and O2 consumption are normal

38. Phygocytic Dysfunction
Diseases
3) Chediak Higashi Syndrome
 Autosomal recessive disorder
 Bacterial infection, hepatosplenomegaly, partial
albinism, CNS abnormility
 Abnormal killing of organisms
 Respiratory burst and O2 consumption are normal

39. Phygocytic Dysfunction
Diseases
4) Lazy Leukocyte Syndrome
 Defective neutrophil chemotaxis
 Neutropenia

40. Compliment mediated
Diseases
 Compliment System
 Opsonization, Chemotaxis, non specific immunity
 Autoimmune diseases

41. Compliment mediated
Diseases

42. Combined humoral and cell
mediated immunity
1) Reticular Dysgenesis
 Rare fatal diseases
 Myloid cell fails to differentiate
 Lack phygocytes, lack T and B cell
 Haematopoiesis
 Child dies

43. Combined humoral and cell
mediated immunity
2) Bare Lymphocyte Syndrome
 Syndrome associated with deficiency of MHC
factor or expression
 Type I syndrome
 Type II syndome

44. Combined humoral and cell
mediated immunity
3) Severe Combined Immunodeficiency Disease
4) Wiskott Aldrich Syndrome

45. Acquired Immunodeficiency
Diseases due to acquired diseases
1) Chromosomal Disorder
2) Infective disorder
3) Neoplastic disorder
4) Connective tissue disorder
5) Physical agent inducer

46. Acquired Immunodeficiency
Diseases due to acquired diseases
6) Other condition
7) Iatrogenic causes

47. Clinical Test
 Evaluation of B Cell
 Evaluation of T cell
 Evaluation of Phygocytic cell
 Evaluation of complement system