Immunodeficiency
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IMMUNODEFICIENCY. By: Oki Suwarsa, dr, MKes, SpKK(K). IMMUNODEFICIENCY.  Risk of opportunist infection & tumors Divided in two types: Primary immunodeficiency : Relatively rare Genetic basis Secondary immunodeficiency: More common Caused by lesion outside the immune system.

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Immunodeficiency

IMMUNODEFICIENCY

By: Oki Suwarsa, dr, MKes, SpKK(K)


Immunodeficiency1
IMMUNODEFICIENCY

  •  Risk of opportunist infection & tumors

  • Divided in two types:

    • Primary immunodeficiency :

      • Relatively rare

      • Genetic basis

    • Secondary immunodeficiency:

      • More common

      • Caused by lesion outside the immune system


Repeated unusual infection immunodeficiency
Repeated/unusual infection  immunodeficiency

  • Type of infection  cause & degree of immunodeficiency

    • Repeated bacterial infection  defective antibody

    • Respiratory infection caused by Pneumococcus or Haemophilus spp.  bronchiectasis

    • Gram - & fungi  abnormal phagocytes

    • Meningococcal meningitis  defective complement

    • Defective T cells or macrophages  infection of intracelullar organisms : protozoa, virus, intracellular bacteria (mycobacteria & salmonella)


  • Reactivation of herpes virus infection  T cell immunodeficiency

  • Herpes virus induce tumors, kaposi’s sarcoma, non-Hodkin’s lymphoma  T cell dysfunction

  • the degree of T cell immunodeficiency  pattern of mycrobial infection

    • Mild  Mycobacterium tuberculosis (virulent) spreads outside the lungs

    • Severe  infection of mycobacterium of low virulence, found in environment (Mycobacterium avium) or vaccine


Features of immunodeficiencies affecting t b lymphocytes
Features of immunodeficiencies immunodeficiencyaffecting T & B lymphocytes


Immunodeficiency caused by defect in b t cells maturation
Immunodeficiency caused by immunodeficiencydefect in B & T cells maturation





Primary immunodeficiency

Infection immunodeficiency

Tumors

Primary immunodeficiency

Gene therapy

Mutation

Polymorphisms

Immunoglobulins

Polygenic

PRIMARY IMMUNODEFICIENCY


Causes of primary immunodeficiency
Causes of primary immunodeficiency immunodeficiency

  • Mutation :

    • Rare, affect any part of immune system

    • Severe disease

  • Polymorphisms :

    • Common traits, affect any part of the immune system

    • Moderate increased risk of infection

  • Polygenic disorders :

    • Relatively common, affect mainly antibody

    • Severe disease



Defects in innate immune system are characterized by extracellular pathogen infections
Defects in innate immune system are characterized by extracellular pathogen infections


Mutations and immunodeficiency
Mutations and immunodeficiency extracellular pathogen infections

  • 12 important mutations  immunodeficiency

  • > Mutation  severe combined immunodeficiency (SCID)

    • Affecting both B & T cells

    • Autosomally inherited  RAG deficiency

    • X linked  γ-chain deficiency & hyper-IgM syndrome

    • The Di George syndrome  translocated of chromosome 22, not inherited


T cell defects
T cell defects extracellular pathogen infections


Polymorphisms immunodeficiency
Polymorphisms & immunodeficiency extracellular pathogen infections

  • Polymorphisms : alleles of the same gene occurring at a single locus in 1% population

  • HLA alleles  polymorphic  affect the outcome of infection (hepatitis B, hepatitis C & HIV)

  • Polymorphisms promoter of TNF genes   risk cerebral malaria & septic shock

  • Polymorphisms chemokines & their receptors   risk HIV

  • Polymorphisms in MBL & complement   risk infections


Polygenic disorder
Polygenic disorder extracellular pathogen infections

  • Caused by interaction of several genes with environmental factors

  • Common variable immunodeficiency (CVID) & deficiency of IgA & IgG  common polygenic disorder, affecting Ab

  • IgA deficiency  1:600 people

  • Celiac disease  more common in IgA deficiency

  • CVID  commonest immunodeficiency  treatment

  • CVID  recurrent infection of respiratory tract, start in early adult

  • Autoimmunity is common in CVID & frequently includes pernicious anemia & thyroid disease, arthritis & immune thrombocytopenia


Diagnosis scid should be considered if
Diagnosis SCID should be considered if: extracellular pathogen infections

  • Unusual or recurrent infection

  • Failure to thrive & diarrhea

  • Unusual rashes

  • A family history of neonatal death or of consanguinity

  • A very low total lymphocyte count (below 1 x 109/L)


  • Avoid serious infection: extracellular pathogen infections

    • Avoid live vaccine : BCG, measles, mumps, rubella & polio

    • Use prophylaxis against opportunist infections : Pneumocystis carinii pneumonia

  • Suspect SCID  exclude HIV  refer to spesialist to confirm diagnosis  definitive treatment (often bone marrow transplant)


Diagnosis
Diagnosis extracellular pathogen infections

  • Chronic or recurrent bacterial respiratory infection  Indication for testing  measure of IgG, IgA & IgM

  • If total Ig normal  measure IgG subclasses & spesific Ab against Haemophilus & Pneumococcus spp.

  • If all test normal  check complement or neutrophil function

  • Patient with atypical viral, protozoal or mycobacterial infection  rule out T cell immunodeficiency

  • Patient with suspected cellular immunodeficiency  measure lymphocyte numbers

  • Genetic testing  PCR


Sepuluh tanda imunodefisiensi
Sepuluh tanda imunodefisiensi extracellular pathogen infections

Duaataulebihgejaladalamsatutahun

Jeffrey Modell Foundation


Empat atau lebih infeksi telinga dalam setahun extracellular pathogen infections

Dua atau lebih infeksi sinus berat dalam setahun

Pada bayi terjadi kegagalan peningkatan berat badan dan gagal tumbuh

Dua bulan atau lebih pemakaian antibiotik dengan sedikit efek

Dua episode atau lebih pneumonia dalam setahun


Kandidiasis persisten di mulut atau di mana saja usia >1 tahun

Abses berulang pada kulit dan organ dalam

Riwayat keluarga dengan imunodefisiensi primer

Kebutuhan penggunaan antibiotik intravena untuk mengatasi infeksi

Dua atau lebih infeksi dalam termasuk septikemia


Treatment
Treatment tahun

  • Aim of treatment  prevent infection

  • Mild cases  prophylactic antibiotics

  • More severe Ab deficiency  immunoglobulin replacement therapy (intravenous or subcutaneus)

  • T cell deficiency  bone marrow transplant (BMT)

  • If BMT isn’t option  gene therapy



Secondary immunodeficiency

vaccines tahun

Infection

Tumors

Immunomodulation

Secondary immunodeficiency

vaccines

HIV

Immunomodulation

Stress

Nutrition

Extremes of age

Drugs

SECONDARY IMMUNODEFICIENCY


Secondary immunodeficiency1
Secondary immunodeficiency tahun

  • Can be severe :

    • HIV infection

    • Myeloma

    • Some drug treatments



Hiv infection
HIV Infection tahun

  • Most important caused of secondary immunodefiency  affecting over 30 million people

  • Monitoring infection :

    • Immunological monitoring  CD4 cell counts  form part of assessment schemes for progression of HIV infection (CDC)

      • CD4 < 200 /l  high risk PCP  prophylaxis

      • CD4 < 100 /l  consider CMV & atypical mycobacteria

    • Virological monitoring  viral load












Other secondary immunodeficiency
Other secondary immunodeficiency tahun

  • Nutrition

    • Deficiency of zinc & magnesium  impairs cell mediated immunity, particularly TH1-cytokine secretion

    • Loss of fat  low levels of leptin  mild immunodeficiency

  • Physiological stress

  • The immune system in the first year of life

    • Spesific immune system remain immature

    • Neonates have high number of T cells  all naive  not respond to Ag

    • Transient hypergammaglobulinemia of infancy  delay in maturation of Ig, especially IgG2 (maternal Ab ↓)


Other secondary immunodeficiency1
Other secondary immunodeficiency tahun

  • The aging immune system

    • Elderly  thymic function ↓  more infection

  • Miscelaneous factors

    • B cell malignancy

      • Myeloma & chronic lymphocytic leukemia  ↓ Ab  common caused of immunodeficiency in elderly

      • Thymoma  rare tumor  cause immunodeficiency


Other secondary immunodeficiency2
Other secondary immunodeficiency tahun

  • Drugs  Common caused

    • Eliminating offending drug  improve immune response

    • Immunosupression  side effect steroids, cytotoxic drugs & immunosupressive regimens

  • Kidney disease

    • Nephrotic syndrome

      • Renal protein loss

      • ↓ blood level of IgG & IgA, normal IgM

    • Severe diarrhea  lost igG via the gut

    • Renal failure & diabetes  secondary phagocyte defect


  • Infection tahun

    • Malaria & congenital rubella  Ab deficiency

    • Measles  defects in cell mediated imunity  could reactivate tuberculosis


Combined immunodeficiency
Combined immunodeficiency tahun

  • Syndrome:


Thank you

Thank You tahun


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