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Understanding Immunodeficiency Disorders: A Comprehensive Overview

This lecture provides an in-depth understanding of immunodeficiency disorders. By the end, participants will comprehend the anatomy and physiology of the immune system, recognize signs of immunodeficiency, and classify various types of disorders. Key topics include the roles of primary and secondary organs of the immune system, the functions of different immune cells, and the laboratory tests essential for diagnosis. Treatment strategies will also be outlined, covering antimicrobial therapy, nutritional support, intravenous immunoglobulin (IVIG), and bone marrow transplantation (BMT).

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Understanding Immunodeficiency Disorders: A Comprehensive Overview

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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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