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AANIYA PATH > Aani Immuno: Primary Immune Deficiencies > Flashcards

Aani Immuno: Primary Immune Deficiencies Flashcards

1
Q

What type of antibodies do immature B cells produce?

A

IgM only

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2
Q

How are IgG, IgA and IgE Abs produced?

A

Following CD4 cells + B cells class switch

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3
Q

What are the types of Primary Immune Deficiencies?

A

T cell
B cell
Phagocyte
Complement

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4
Q

What are the types of Deficiencies that affect T cells?

A

Bare Lymphocyte Syndrome
DiGeorge Syndrome
SCID (mixed B and T)

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5
Q

What are the types of Bare Lymphocyte Syndrome?

A

Type 1 and Type 2.
Type 1 = Low MHC Class 1
Type 2 = Low MHC Class 2

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6
Q

Which type of Bare Lymphocyte Syndrome is more common?

A

Type 2

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7
Q

Describe the cells of Type 2 Bare Lymphocyte Syndrome?

A

It would have NORMAL CD8 and B cells. But absent CD4 cells.
This means that the germinal centre reaction between B cells and CD4 cannot take place so IgM can be made by B cells but IgG/IgA and IgE cannot be made.

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8
Q

Which condition is associated with Bare Lymphocyte Syndrome?

A

Sclerosing Cholangitis

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9
Q

What is the chromosomal defect in DiGeorge Syndrome?

A

22q11.2 deletion

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10
Q

What happens in DiGeorge Syndrome (in terms of T cells)?

A

Impaired development of 3rd and 4th pharyngeal pouch, absent thymus so T cells cannot mature = low mature T cells.

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11
Q

What are the clinical features of DiGeorge Syndrome?

A 
CATCH 22 
Cleft palate 
Abnormal facies - low ears, high forehead 
Thymic aplasia 
Cardiac abnormality - tof 
Hypoparathyroid

CHD (because aortic arch is alo made from 3/4 pharyngeal pouch)

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12
Q

Treatment of DiGeorge Syndrome?

A

Infection prophylaxis
Thymus transplant
Ig replacement

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13
Q

Treatment of Bare Lymph Syndrome type 2?

A

BMT
Infection prophylaxis
Ig replacement

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14
Q

What are the types of B Cell primary Immune Deficiencies?

A 
Bruton's Agammaglobulinaemia
Common Variable Immune Deficiency
Selective IgA deficiency 
Hyper IgM Syndrome 
SCID (mixed B and T cell)
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15
Q

Which Igs are missing in Bruton’s?

A

ALL because no B Cells

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16
Q

Which Igs are missing in Common Variable Immune Deficiency?

A

IgA, IgG and IgE (basically the 3 ones that mature B cells are needed to make)

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17
Q

Which Igs are missing in Selective IgA deficiency?

A

IgA lol

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18
Q

What causes the Ig defect in Common Variable Immune Deficiency?

A

Failure of B cell differentiation

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19
Q

How to tell the diff between Common Variable Immune Deficiency and Brutons?

A

Common Variable will have IgM. Brutons wont have any Ig.

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20
Q

Which is the most common B Cell Immune Deficiency?

A

Selective IgA deficiency

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21
Q

Who Gets Bruton’s?

A

Boys - it’s X linked.

Presents between 3-6 months

22
Q

What happens in Hyper IgM syndrome?

A

T cells and B cells cannot interact to class switch so IgA and IgG are not made. IgM are elevated

23
Q

What is SCID?

A

Severe Combined Immune Deficiency. Impaired B and T cell development

24
Q

Tx for SCID?

A

BMT

25
Q

Mnemonic for remembering B Cell immune deficiencies?

A

Brutus - Brutons
Caesar - combined Variable
Soccrates - Selective IgA def
Hippocrates - HyperIgM

26
Q

How to categorise the phagocyte deficiencies?

A

Low production
Low migration
Low killing

27
Q

What are the Phagocyte Deficiences?

A

Kostmann
Cyclic Neutropenia
Leukocyte adhesion deficiency
Chronic Granulomatous Disease

28
Q

What is Kostmann Syndrome?

A

Congenital neutropenia - failure of neutrophil maturation (HAX1)

29
Q

What is Cyclic Neutropenia?

A

Low neutrophils every 3 weeks. Lasts 6 days

mutation in neutrophil elastase

30
Q

What is Leukocyte Adhesion Deficiency?

A

Low Leukocyte adhesion molecules due to B2 integrin mutation on subunit CD18

no cd18/11a means can’t latch on to endothelial cells to get into tissue.

no pus, delayed umbilical cord separation

31
Q

What is Chronic Granulomatous Disease?

A

NADPH Oxidase deficiency. Leading to poor oxidative killing of organisms. Patients susceptible to catalase positive organisms (PLACESS) Psuedo, Listeria, Aspergillus, Candida,E.Coli, Staph A

granulomas form bc can’t kill the bugs
lymphadenopathy, hepatosplenomeglay

32
Q

How do you investigate for Chronic Granulomatous Disease?

A

The colour change test and fluorescent test will be negative (NBT and DHR)

nitroblue tetrazolium
dihydrorhodamine

33
Q

What is Reticular Dysgenesis?

A

A very severe form of SCID where you have absolute deficiency in Neutrophils, Lymphocytes, Platelets, Monocytes

34
Q

Why can you get secondary complement deficiency from SLE?

A

SLE immune complexes are removed by classical pathway and so the complement gets used up and you get deficiency

35
Q

What are the stages of the complement cascade?

A
  1. Classical pathway
  2. Lectin Pathway
  3. Alternative Pathway

They all activate C3, therefore C5

36
Q

Which test do you do for Classical Pathway deficiency?

A

CH50 = will be abnormal

CH50 test is for C1,C2,C4,C3,C5-C9

37
Q

What microorganisms are more likely to infect someone with a complement deficiency?

A

Encapsulated e.g. Niessera Meningitidis, Group B Strep

38
Q

Which molecules involved in Classical pathway?

A

C1,C2,C4

39
Q

Which molecules are involved in Alternative Pathway?

A

B,I,P

40
Q

Which test do you do for Alternative pathway deficiency?

A

AP50 = will be abnormal

AP50 is for B,I,P,C5-C9

41
Q

What can be wrong in the Lectin Pathway?

A

Mannose Binding Lectin deficiency

42
Q

Which tests will be abnormal in a defect with the common/terminal complement pathway?

A

Both CH50 and AP50 because they both include tests for C5-C9

43
Q

How can glomerulonephritis affect complement?

A

Nephritic factor causes constant activation of C3. so there is low C3

44
Q

Meningococcal infections are quite common as a result of which deficiency of the component of the immune system?

A

Complement

45
Q

Which infection is most common as a consequence of B cell deficiency?

A

Bacterial

46
Q

Kostmanns syndrome is a congenital deficiency of which component of the immune system?

A

Neutrophil phagocytes

47
Q

Which component of the innate immune system is usually one of the first to respond to infection through a cut?

A

Neutrophil phagocytes

48
Q

Which complement factor is an important chemotaxic agent?

A

C3a

49
Q

What is the functional complement test used to investigate the classical pathway?

A

CH50

50
Q

Which Crucial enzyme is vital for the oxidative killing of intracellular micro-organisms?

A

NADPH oxidase

51
Q

Leukocyte Adhesion Deficiency is characterised by a very high count in which molecule?

A

Neutrophils

52
Q

A complete deficiency in this molecule is associated with recurrent respiratory and gastrointestinal infection?

A

IgA

AANIYA PATH (35 decks)

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