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Immunology > Primary Immunodeficiency > Flashcards

Primary Immunodeficiency Flashcards

1
Q

What are the 2 types of immune system

A

Innate

Adaptive

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2
Q

What does the immune system start, describe the process

A
  1. Danger signals (PAMPs and DAMPs) in the tissue are recognised by antigen presenting cells
  2. Antigen presenting cells present the antigen to CDT tell cells
  3. CD4 cells give instructions to b cells
  4. B cells produce antibodies which act as opsonins and activate complement
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3
Q

What is the role of b cells

A

Produc antibodies

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4
Q

What are the the types of t cells and what are the functions

A

Cd4 t cells: produce cytokines to support b cells
Cd8 t cells: cytotoxic cells that destroy virally affected cells and tumour cells
T reg cells: help suppress auto reactive t cells

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5
Q

What is the role of phagocytes

A

Engulf and destory extracellular pathogens and present antigens

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6
Q

What is the role of complement

A

Opsonination (c3b)
Immune complex clearance (c1q)
Membrane attack complex

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7
Q

What is the role of nk cells

A

Destroy virally infected cells and tumour cells

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8
Q

When should we consider immunodeficiency

A

When we see recurrent infections

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9
Q

Where could the defect be in immunodeficiency

A 
Non immune defect e.g non Anatomical structure problem
T cell defect
B cell defect
Combined defect of t and b cells
Innate defect (neutrophils and complement problems)
Global defect (signalling molecular and cytokines)
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10
Q

What are the 4rs in the immune response

A
  1. Problem in the recognition of pathogen
  2. Probelm in the response to pathogen
  3. Problem in the regulation of immune response
  4. Problem in generation resolution and memory
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11
Q

What makes you think of immunodeficiency

A

Severe Recurrent invasive infection
Unusual organism or site of infection
Severe recurrent or disproportionate inflammation
Unexplained lymphoproliferation

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12
Q

If a baby has lymphocytes count less than 1 what does this suggest if unless proven otherwise

A

Severe combined immunodeficiency

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13
Q

Where do normal lymphocytes come from

A
  1. Common lymphoid precursor differentiate into b cell, t cell and nk cell precursors
  2. B cell precursor turn into naive b cells
  3. T cell precursor turn into t naive cell
  4. Nk cell precursor turn into nk cells
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14
Q

What can stop this differentiation of lymphocytes from occuring

A

Genetic disease

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15
Q

What is ADA SCID

A

Leads to build up toxic metabolite in the common lymphoid precursor that causes it to die. You therefore get negative t, negativ b and negative nk cell SCID.

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16
Q

What does genetic disease that causes tcr rearrangement failure in t cell and b cell precursor lead to

A

B negative, t negative and nk cell positive SCID

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17
Q

What does t cell lineage specifc loss of survival signals lead to

A

T cell negative, b cell positive and nk cell positive SCID

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18
Q

Wgat does cell sruvival signal failure in t cell precursor and nk cell lead to

A

T cell negative, nk cell negative and b cell positive SCID

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19
Q

What is the presentation of SCID

A

Early onsent of infection that fail to clear
Child fails to thrive
Erythrodemic rash- caused by mothers lymphocytes attacking the baby
Eosiniophillia
Lymphopenia

20
Q

What is the management of SCID

A

Initiate prophylactic antifungal, antibiotics, antiviral and IVIG
Strict infection control
No live vaccine or breast feeding
CMV negative and irradiarted blood production to prevent engrafment of lymphocytes

21
Q

What is the aim of the management of SCID

A

To get child into a position for bone marrow transplant

22
Q

What are the function of antibodies

A

Opsonistation
Antibody dependent cytoxicityt with NK cells
Phagocytosis
Neutralise
Agglutinate pathogen to make them easier to phagocytosis

23
Q

If there is antibody deficiency what are the types of infections you can get

A

Pneumonia
Bronchiectesis
Conjunctivitis
Otitis media

24
Q

What is the characteristic pattern of pathogen that you can get in antibody deficiency

A

Streptococcal infection
Haemophillus infection
Staphyloccocal infection
Giardiasis, campylobacter, salmonella

25
Q

What are the genetic causes of antibody deficiency

A

Brutons tyrosine kinase in boys
X linked agammaglobulinemia
Hyper igm syndrome

26
Q

What is brutons tyorsine kinase

A
  1. Btk is a signalling molecule that helps be cells keep alive as they rearrange from pro b to pre b cells
  2. Without BTK there is be cell maturation arrest so you are unable to make naive b cells
  3. No naive b cells means no antibodies
27
Q

What is x linked agammaglobulinaemia (XLA)

A

X linked disease where there are absent b cells and undetectable immunoglobulin

28
Q

What is the treatment of xla

A

Prophylactic antibiotics
Replacement of immunoglobulin
Respiratory opionin

29
Q

What is hyperigm syndrome

A

When b cell development needs to cell help

30
Q

To swtich from igm to igg what need to happen

A
  1. Antigen binds to b cells
  2. Antigen is internalised and put onot the surface of b cells as MHC
  3. T cells recognise MHC
  4. This causes class swtich where igm is switched to igg in b cells
31
Q

What causes hyper igm syndrome

A

A deficiency in CD40 ligand on t cells leads to inability to generate the signal needed to class swith so there is high igm

32
Q

What are the types of neutrophil defects

A

Defect in the neutrophil number
Defect in neutrophil chemotaxis- the way neutrophils migrate in blood to tissue
Defects in neutrophils killing

33
Q

What is x linked chronic granulomatous disease

A

A defect in neutrophil killing

34
Q

What is the pathogeneis of chronic granulomatous disease

A
  1. Failure of the one of the subunit of NADPH complex
  2. Purpose of the NADPH complex is to produce ROS which then generates a potential difference in the vacuole
  3. Build up of potential difference causes pottasium influx into the vacuole that causes activation of enzymes to kill of ingested bacteria
  4. Failure of NADPH complex means you cant kill the bacteria
35
Q

What is the tratment of chronic granulomatous disease

A

Prophylactic antibodies
Antifungals
Gene therapy

36
Q

How does chronic granulomatous disease present

A

Early onset deep seated infection with catalase positive organism, staph, aspergillus, norcardia, serratia

IBD like diease

37
Q

What is the complement cascade involved in

A

Classical pathway via antibody
Mannose binding lectin pathway
Alternative pathway

38
Q

What do 3 pathways activate

A

Cause c3 to cleave into c3a and c3b

39
Q

What does c3 do

A

Cleave c5

40
Q

When c5 is cleaved what ahppens

A

TCC is assembled on the surface of the antigen of the pathogen to help punch a hole through it

41
Q

What is a deficiency associated with the classical pathway

A

Lupus like disease

42
Q

What is a disease associated with the MBL deficiency

A

Usually asymptomatic

43
Q

What is a disease associated with the alternative pathway

A

Neisseria meningitis

44
Q

What disease is a defect in terminal complement complex (TCC) lead to

A

Neisseria meningitis

45
Q

What is common variable of immunodeficiency (CVID)

A

Hypogammaglobulinemia (low igg, iga,igm): causes recurrent infections such as sinupulmonary
Polyclonocal lymphoproliferation: causes large lymph nodes, spleen
Autoimmunity: causes vitiligo, hypothyroid
Granulomatous disease: gi granulomata
Enteropathies: chronic norovirus, IBD

46
Q

What is the management of CVID

A

Immunoglobulin replacement
Prophylactic antibodies
Immunosupresssion
Bone marrow transplant

Immunology (7 decks)

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