Immunodiffeciency Disesases. pptx

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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 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 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 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 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 What are the means by which antibody molecules exert a protective effect? Billy was free of infections for the fi rst few months of life. Why? Why did Billy lack tonsils? 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 O 2 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 O 2 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 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 Chromosomal Disorder Infective disorder Neoplastic disorder Connective tissue disorder Physical agent inducer

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

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