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Immunodeficiency .


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primary and secondary immune deficiencies

primary and secondary immune deficiencies

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  • 1. Immunodeficienc y Fawzia aboaliP of internal mdicine & clinical immunology rof Ain shams faculty of medicine
  • 2. Objectives: immune system Immune deficiency,classifications. Immune defect & organisms Primary immune deficiency Secondary immune deficiency:types Diagnosis of immune defects Management
  • 3. The 4 Arms of Immune System & Theircommand
  • 4. ImmunodeficiencyDefinitionImmunodeficiency (or immune deficiency) is a state in which the immune system s ability to fight infectious disease is compromised or entirely absent. Immunodeficiency may also decrease cancer immunosurveillance.
  • 5. Primary Immunodeficiencies Stem Cell Myeloid Lymphoid Progenitor Progenitor Severe combined Congenital Immunodeficiency Agranulocytosis SCID Monocyte Pre-B Pre-TNeutrophil x-linked aγglobulinemia xLA Mature B Thymus DiGeorge Syndrome d Mature Plasma T Cell Memory BCommon Variable HypoγglobulinemiaSelective Ig deficiency
  • 6. IMMUNE DEFICIENCY OPPORTUNISTIC INFECTIONS: Opportunistic infections are mild to severe infectious diseases in a compromised host. The infections are caused by microorganisms that normally do not cause serious disease in healthy people. Viral, Bacterial, Fungal, Protozoan, Helminthic Opportunistic Malignancies: Kaposis Sarcoma and Lymphomas
  • 7. T h e  1 0  w a r n i n g  s i g n s  o f   i m m u n e d e f i c i e n c y
  • 8. Common variable Immunodeficiency (CVI).Heterogynous group that cause late - onset hypogammaglobulinaemia.Recurrent infections between 15 - 35 yrs.Features:1.Low IgG & IgA .2.Impaired antibody responses.3.Associated autoimmune diseases•Recurring infections involving the ears, eyes,sinuses, nose, bronchi, lungs, etc.•The organisms commonly found in these infectionsare bacteria that often cause pneumonia(Haemophilus influenzae, pneumococci, andstaphylococci).
  • 9. IgA deficiency (1:700) Most are asymptomatic. ( but haveincreased rate of (R.T.I.) Some have recurrent R.T.I and G.I.T. symptoms Increased incidence of allergic manifestations. anti- convlusant drugs (phenytoin) may cause deficiency .
  • 10. Common Variable Immunodeficiency (Late- Onset Hypogammaglobulinemia) Onset usually in 2nd to 4th decade of life Slow decline in all classes of immunoglobulin Recurrent sinopulmonary infections (usually bacterial in origin) Gastrointestinal, endocrine, hematologic and autoimmune disorders can be associated May follow Epstein-Barr infection Increased incidence of lymphoreticular malignancies
  • 11. David Phillip Vetter (September 21,1971 – February 22, 1984)
  • 12. SECONDARY IMMUNE DEFICIENCY1. Acquired Immune Deficiency Syndrome (AIDS)2. Cancer / Chemotherapy:3. Immunosuppression In Diabetes4. Immunosuppression In Transplant Pts5. Autoimmune disease6. Immunosuppression Related to Steroid Use7. Immunosuppression In Asplenic Pts8. Effect of Aging On Immune Competence9. pregnancy
  • 13. 1. Acquired Immune Deficiency Syndrome (AIDS)a) Etiology& presentationb) Defect: Inversion of T helper/inducer cells (OKT4) to cytotoxic/suppressor cell (OKT8) ratio Normal T4/T8 = 2/1 In AIDS it is 0.5. 
  • 14. CD4 Count Greater than 500 / ul - Almost normal defense mechanisms Less than 200 / ul - Opportunistic AIDS related infections Less than 100 / ul - Life threatening complications
  • 15. Opportunistic Infections in AIDS Patients
  • 16. 2. Cancer / Chemotherapy: Unfortunately in cancer, both the disease and the treatment can cause immunosuppression : Neutropenia Cell Mediated Immunity Humoral factors
  • 17. Neutropenia Occurs approximately two weeks after the last dose of chemotherapy an absolute neutrophil count of less than 1,000 cells/mm3 on the way down are at increased risk for a serious bacterial infection.Management strategy:1. All patients with anticipated severe neutropenia (ANC < 0.5 x 109/l), should receive prophylaxis against bacteria, fungi,viruses&G-CSF2. Neutropenic patients who present with fever require a prompt switch to an appropriate treatment regime
  • 18. Cyclic neutropenia is a form of neutropenia that tends to occur every three weeks and lasting three to six days at a time due to changing rates of cell production by the bone marrow. It is often present among several members of the same family. Treatment includes G-CSF and usually improves after puberty.
  • 19. 3. Immunosuppression In Diabetes Humoral Immunity: normal Ab levels & vaccination responses. Impaired Cellular Immunity: Impaired Innate Cellular Defenses:o PMN abnormalities - adherence, chemotaxis,o Phagocytosis.Infections In Diabetics rhinocerebral mucormycosis oral &esophageal candidiasis surgical / wound infection T.B
  • 20. GSACEP © 2005
  • 21. 4. Immunosuppression In Transplant Pts Infection 2nd most impt problem posttransplantationPre-transplant Host Factors: ongoing medical conditions (HBV, HCV, diabetes) prior MO colonization ( Candida, staph) prior latent infection (TB, CMV) prior medications (i.e. immunosuppressives,antibiotics)Common Microbial Etiologies Post-Transplantation Bacteria: common gm+ & gm- flora Fungi: Candida sp.; Aspergillus sp
  • 22. 5. Immunosuppression in autoimmune Diseases:Multiple infection risk factors:1. functional defect in phagocytic cells.2. CMI defects: lymphopenia, CD4 - cell3. reduced Ab levels & low complement levels.4. functional asplenia.5. also, corticosteroids & immunosuppressivesincrease infection risks.
  • 23. 6. Immunosuppression Related to Steroid Use Glucocorticoids (corticosteroids) have inhibitory effects on T cells and B cells, as well as phagocytes. Infections depend on route of administration, dose, & duration of therapy.
  • 24. Infections Related to Steroid Use increased susceptibility to all types ofinfection. Prolonged CMI suppression important foropportunistic infection to occur. Fever may be absent delayed wound healing & wound infections:steroids interfere with fibroblast proliferation & collagen synthesis.
  • 25. 7. Immunosuppression In Asplenic Pts• lower C3 levels & defective responses to encapsulated bacterial pathogens• decreased phagocytosis• failure to recognize polysaccharide Ag’s.• impaired IgM synthesis early in infection.Pathogen:-S. pneumoniae ,H influenzae ,N. meningitidis Sickle Cell Functionally aspenic (ask about immunizations!)
  • 26. 8. Effect of Aging On Immune Competence Declining :Innate, Humoral & Cellular ImmuneResponses Increased Susceptibility to Pneumonias & Chronic Infections
  • 27. During pregnancy:
  • 28. Initial Evaluation of Possible Immunodeficiency Make sure that what seems to be infections are not really: ATOPY, ALLERGY, ASTHMA Exclude other conditions Hold off on any live viral vaccines or transfusions until situation well defined Document that there have been multiple infections Look for other, non-immune features of immunodeficiency: rash, hypocalcemia, facial characteristics Family history
  • 29. Initial screening tests for immunodeficiency CBC: WBC,function Quantitative immunoglobulins: IgG, A & M Total lymphocyte count T-cell enumeration, with subsets CD4, CD8 Evaluate for current infections: CULTURES, ESR, CRP, X-RAYS.
  • 30. Tests for B cell,antibody deficiency: Total ,lymphocyte count Total serum immunoglobulins IgG subclasses, Antibodies for pervious vaccination Immunoglobulin function& survival Tests for cellular deficiency: Total lymphocyte count T-cell enumeration, with subsets CD4, CD8 Functional assays: antigens response to mitogens, cytokines assay. Delayed hypersensitivity reaction for Tuberculin and Candida antigen
  • 31. Tests for other deficiency:Phagocyte:i. Neutrophil countii. NBT test for screening.Complement:Total and specific complement count.
  • 32. Treatment options monitor Live vaccines are absolutly contraindicated
  • 33. 1. IVIG .( IV infusion of immunoglobulin.) For : a. agammaglbulinaemia . b. CVI. c. WAS2. Periodic antibiotic treatment.3. Bone marrow transplantation . For : a. SCID . b. WAS.4. Enzyme replacement . For : ADA deficiency.5-CSF.(colony stimulating factor ) For : neutropenia6. Thymus transplantation . For : DiGeorge syndrome.7. IFN – gamma . For : CGD.8-gene therapy
  • 34. 1) Which one of the following does 3) Which of the following not predispose to superficial tests may assess Candida albicans infection? cellular immune A   Pregnancy B   Lymphoma dysfunction; C   Diabetes mellitus a)    CD4, CD8. D   Vegetarian diet b) Total serum2)  Which of the following is not commonly associated with immunoglobulins marked secondary antibody deficiency? c) IgG subclassesa) Multiple myeloma. d) Immunoglobulinb)  autoimmune diseases.c)  HIV infection. responsed)  hypersplinism.