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ranjithakm-180315143057.pdf

  1. 1. WELCOME
  2. 2. IMMUNODEFICIENCY RANJITHA K.M. PGS15AGR6842 Ranjitha K.M. PGS15AGR6842
  3. 3. Content  Introduction  Classification  B celldeficiency  T Celldeficiency  SCID  Secondaryimmunodeficiency  TreatmenttoIDs WHAT WILL I LEARN TODAY?
  4. 4. What is immunodeficiency?  Immunodeficiency (or immune deficiency) is a state in which the immune system's ability to fight infectious disease is compromised or entirely absent.
  5. 5. Classification  Primary immunodeficiency  Secondary immunodeficiency
  6. 6. Primary immunodeficiency  Defects in genes for components of the immune system.  Victims are born with these diseases, which are the result either of inherited or developmental defects.  Cause: mutations in genes involved in the development and function of immune organs, cells, and molecules.
  7. 7. Secondary immunodeficiency  Due to factors that have an adverse impact on the immune system.  These are acquired as secondary results of various disease states, due either to the disease processes themselves or the therapy used to treat them.  Loss of previously functional immunity due to infection, toxicity, radiation, splenectomy and malnutrition
  8. 8. Four Categories Immune Mechanisms  Primary immunodeficiency Humoral (Antibody or B-cell mediated) Cell-mediated (T-cell mediated) Complement system Phagocytosis
  9. 9. B CELL DEFICIENCY  X liked a gammaglobuinemia  Early maturation of B cells fails,  Few or no B cells in blood  Very small lymph nodes and tonsils  No Ig,Small amount of Ig G in early age  Recurrent pyogenic infection  IgA and IgG subclass deficiency  IgA deficiency is most common  Patients tend to develop immune complex disease  About 20% lack IgG2 and IgG4  Susceptible to pyogenic infection  Result from failure in terminal differentiation of B cells
  10. 10.  Immunodeficiency with increased IgM  Results in patients with IgA and IgG deficiency  Production of large amount of IgM >200mg/dl of polyclonal IgM  Susceptible to pyogenic infection  Treatment by iv gamma globulin  Formation of IgM to neutrophils, platelets and other blood components  Transient hypogammaglobulinaemia of infancy  Due to delay in in IgG synthesis approximately up to 36 months  In normal infants synthesis begins at 3 months  Normal B lymphocytes  Probably lack help of T lymphocytes
  11. 11.  Common variable immundeficiency  There are defect in T cell signaling to B cells  Acquired a gammaglobulinemia in the 2nd or 3rd decade of life  May follow viral infection, Pyogenic infection  80% of patients have B cells that are not functioning  B cells are not defective. They fail to receive signaling from T lymphocytes
  12. 12. T cell deficiencies 1- DiGeorge's syndrome: o It the most understood T-cell immunodeficiency o Also known as congenital thymic aplasia/hypoplasia o Associated with hypoparathyroidism, congenital heart disease, fish shaped mouth. o Defects results from abnormal development of fetus during 6th-10th week of gestation when parathyroid, thymus, lips, ears and aortic arch are being formed
  13. 13. 2- Ataxia-telangiectasia  Associated with a lack of coordination of movement (ataxis) and dilation of small blood vessels of the facial area (telangiectasis).  T-cells and their functions are reduced to various degrees.
  14. 14. 3- Wiskott-Aldrich syndrome  Associated with normal T cell numbers with reduced functions, which get progressively worse.  IgM concentrations are reduced but IgG levels are normal  Both IgA and IgE levels are elevated.  Boys with this syndrome develop severe eczema.  They respond poorly to polysaccharide antigens and are prone to pyogenic infection.
  15. 15. MHC DEFICIENCY (Bare leukocyte syndrome):  Due to defect in the MHC class II transactivator (CIITA) protein gene, which results in a lack of class-II MHC molecule on APC.  Patients have fewer CD4 cells and are infection prone.  There are also individuals who have a defect in their transport associated protein (TAP) gene and hence do not express the class-I MHC molecules and consequently are deficient in CD8+ T cells.
  16. 16. Defects of the phagocytic system Defects of phagocytic cells (numbers and/or functions) can lead to increased susceptibility to a variety of infections. 1- Cyclic neutropenia: It is marked by low numbers of circulating neutrophil approximately every three weeks. The neutropenia lasts about a week during which the patients are susceptible to infection. The defect appears to be due to poor regulation of neutrophil production. 2- Chronic granulomatous disease (CGD):  In majority of patients with CGD, the deficiency is due to a defect in NADPH oxidase that participate in phagocytic respiratory burst.
  17. 17. Disorders of complement system:  Complement abnormalities also lead to increased susceptibility to infections.  There are genetic deficiencies of various components of complement system, which lead to increased infections.  The most serious among these is the C3 deficiency which may arise from low C3 synthesis or deficiency in factor I or factor H.
  18. 18. SEVERE COMBINED IMMUNODEFICENCY  In about 50% of SCID patients the immunodeficiency is x-linked whereas in the other half the deficiency is autosomal.  They are both characterized by an absence of T cell and B cell immunity and absence (or very low numbers) of circulating T and B lymphocytes.  Patients with SCID are susceptible to a variety of bacterial, viral, mycotic and protozoan infections.
  19. 19.  The x-linked SCID is due to a defect in gamma-chain of IL-2 also shared by IL-4, -7, -11 and 15, all involved in lymphocyte proliferation and/or differentiation.  The autosomal SCIDs arise primarily from defects in adenosine deaminase (ADA) or purine nucleoside phosphorylase (PNP) genes which results is accumulation of dATP or dGTP, respectively, and cause toxicity to lymphoid stem cells
  20. 20. Secondary immunodeficiencies • Acute and chronic viral infections – infectious mononucleosis, influenza • Metabolic disorders – diabetes mellitus, uremia • Autoimmune diseases – autoantibodies against immunocompetent cells (neutrophils, lymphocytes); autoimmune phenomena also after administration of certain drugs (e.g. oxacilin, quinidine) • Chronic GIT diseases • Malignant diseases (leukemia) • Hypersplenism/asplenia • Burn, postoperative status, injuries • Severe nutritional disorders • Chronic infections • Ionizing radiation • Drug induced immunodeficiencies (chemotherapy) • Immunosupressive therapy • Chronic stress • Chronic exposure to harmful chemical substances
  21. 21.  Severe protein-calorie malnutrtion is primarily associated with a depression of cell-mediated immunity. Ex: kwashiorkor  Deficiencies of pyridoxine, folic acid, and vitamin A are usually associated with cellular immunodeficiency.  Pantothenic acid deficiency is usually associated with a depression of the primary and secondary humoral immune responses.  Vitamin E deficiency is associated with a combined immunodeficiency.
  22. 22.  Burn-Associated Immunodeficiency. Bacterial infections are a frequent and severe complication in burn patients, often leading to death.  Postsurgery immunodeficiency: Any type of surgery represents an acute trauma that can reduce the antiinfectious defenses in a variety of ways  Immunosuppression Associated with Infections  Immunosuppression Associated with Drug Abuse.  The Acquired Immunodeficiency Syndrome (AIDS)
  23. 23. When to suspect immunodeficiency ?  Very frequent infections  Very severe infections  Family history  Unusual microorganism at unusual site  Recurrent respiratory tract infections  Severe bacterial infection  Persistent infection with incomplete response  Persistent sinusitis/mastoiditis  FTT/Growth retardation  Diarrhea/malabsorption
  24. 24. Treatment options incude:  Supportive therapy- antibiotics  Replacement therapy-IV immunoglobulin infusion, ADA-rich RBC infusions  Definitive therapy- Fetal thymic grafts, bone marrow transplantation  Stem-cell therapy  Gene therapy
  25. 25. THANK YOU

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