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Immunodefeciency disorders

Immunodefeciency disorders. DR.FATMA ZAHRANI. Immunodef. (con,d). Objectives: By the end of this lecture you should : 1.know the anatomy& physiology of immune system. 2.know when to suspect immunodef.

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Immunodefeciency disorders

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  1. Immunodefeciency disorders DR.FATMA ZAHRANI

  2. Immunodef. (con,d) • Objectives: By the end of this lecture you should : 1.know the anatomy& physiology of immune system. 2.know when to suspect immunodef. 3.know the classification of immunodef. 4.give example of each type. 5 know the lab.tests to diagnose. 6.outline the treatment of different types.

  3. Immunodef. • Organs of immune system: primary Bone marrow Thymus Secondery Lymphoid tissues Spleen

  4. Immunodef. • Cells of immune system: 1. - B&T cells.(bone marrow&thymus) - Natural killer cells 2.Mononuclear phagocyte (RES). 3.Denteritic cells ( Langerhans cell ). 4.Graulocytes (nutrophils,esinophil –etc)

  5. Immunodef. • Immune response: Bacteria----complement+phagocyte ↓ denderitic lymph node T cell area killed,diff. B cell area plasma,memory

  6. Immunodef. • When to suspect immunodef. Recurrent infection. Delayed response to anti microbial. Opportunestic infection. Failure to thrive. Dysmorphic features. +ve family history.

  7. Immunodef. • Classification : Primary: 1.B cell defect : age 6months+ encapsulated bacteria. sinopulmonary disease. eg: X-linked agammaglobulinemia. selective IgA def

  8. Immunodef. • Primary: 2. T-cell defect: from early life. opportunestic bacteria. Mycobacteria. viruses. chronic diarrhea. malabsorption dysmorphism

  9. Immunodef. • T cell def. Digeorge syndrome Thymus aplasia. parathyroid aplasia vertebral anomalies. cardiac anomalies. dysmorphism

  10. Immunodef. 3.SCID. 4.Phagocytic killing( CGD). chemotaxis. 5.complement.

  11. Immunodef. • Secondry : HIV. Malignency. medication.

  12. Immunodef. • Diagnosis: B.cell defect: Total IgG &subclasses. specific ab. T cell defect: wbc. T cell subset. skin test

  13. Immunodef. • Diagnosis: Nbt. Phage test. CH50 Complement .

  14. Immunodef. • Treatment: • Aggressive parenteral anti microbial. • Nutretional support. • IVIG. • G-CSF. • BMT. • Antiviral .

  15. Immunodef. • Classification: Primary Antibodies def. Cell mediated def. Combined def. Phagocytic dysfunction. Complement def. Secondery .Infection Malignency Medication

  16. Immunodef. • Primary antibodies def. • S&S: age :after 6 months of age. +ve family history. male (Bruton,s disease) sinopulmonary disease. more frequent prolonged even with treatement hepatosplenomegaly +&- lymphadenopathy.

  17. Immunodef. • Bruton disease:

  18. Immunodef. • Transient infantile hypogammaglobulinemia:

  19. Immunodef. • Dignosis: 1. Total immunoglobulins—less than o.5g/dl IgG (total &subclasses) IgM,IgA &? IgE. 2.B-cell markers (CD 3.Isoheamagglutenins. 4.

  20. Immunodef. • Treatement: Regular IVIG. Q4weeks. Aggressive parenteral antimicrobial. Nutritional supports. BMT

  21. Immunodef. • Cell mediated immunodef. S&S: age from neonatal period.candidasis no sex prediliction. ? Family history (Mucocutaneous candidiasis early deathes. ?dysmorphism (DiGeorge) allopecia,nail dystrophy,FTT no BCG scar

  22. Immunodef. • Digeorge syndrome: Sporadic,3rd&4th arches disease. Dysmorphic cardiac,vertebral anomalies sever hypocalcemia(parathyroid aplasia) convulsion thmic aplasia

  23. Immunodef. • Dignosis; • cbc lymphopenia. • no reaction to intradermal test. • CD markers • CXR

  24. Immunodef. • Treatement: • aggressive antimicrobial therapy. • thymus transplantaion • prognosis poor

  25. Immunodef. • Sever combind immundef.:

  26. Immunodef. Phagocytic def. • S&S: age any age. +ve family history (CGD) recurrent suppurativ infection (skin,bone,sinopulmonary). lymphadenopathy. hepatosplenomegaly.

  27. Immunodef. • Diagnosis: • cbc wbc(high OR low) • NBT • phagotest • other

  28. Immunodef. • Treatment: • agrressive antimicrobial . • WBC transfusion • colony stimulation factors. • BMT

  29. Immunodef. • Complement def. • S&S: rare recurrent neisseria infection • Dignosis: CH50 complement assay.

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