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DEFNE Extra Buzzwords > Immunopathology Buzzwords > Flashcards

Immunopathology Buzzwords Flashcards

1
Q

Autosomal recessive severe SCID with no production of lymphoid or myeloid cells

A

Reticular dysgenesis

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2
Q

three examples of failure of neutrophil production

A

Reticular dysgenesis
Kostmann syndrome
Cyclic neutropaenia

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3
Q

Autosomal recessive congenital neutropenia

A

Kostmann syndrome

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4
Q

Autosomal dominant episodic neutropaenia

A

Cyclic neutropaenia

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5
Q

Cyclic neutrophaenia occurs every ? days

A

21 days

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6
Q

inheritance pattern Kostmann syndrome

A

AR

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7
Q

Inheritance pattern cyclic neutropaenia

A

AD

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8
Q

Inheritance pattern Reticular dysgenesis

A

AR

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9
Q

phagocyte deficiency caused by failure of phagocyte migration

A

Leukocyte adhesion deficiency

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10
Q

Leukocyte adhesion deficiency, deficiency of what?

A

Caused by deficiency of CD18 (β2 integrin)

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11
Q

What does CD18 do?

A

CD18 normally combined with CD11a to produce LFA-1 (lymphocyte function associated antigen 1)

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12
Q

Where do leukocytes bind to on endothelial cells?

A

CD18 normally combined with CD11a to produce LFA-1 (lymphocyte function associated antigen 1)
LFA-1 normally binds to ICAM-1 (intercellular adhesion molecule 1) on endothelial cells to mediate neutrophil adhesions and transmigration

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13
Q

Diagnose:
- V high neutrophil count in blood
- Absence of pus formation
- Delayed umbilical cord separation

A

Leukocyte adhesion deficiency

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14
Q

Nitroblue-tetrazolium neg (doesnt change from yellow to blue)

A

Chronic granulomatous disease

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15
Q

Bacterial susceptibility in chronic granulomatous disease

A

Catalase positive - PLACESS
Pseudomonas, listeria, aspergillus, candida, E. coli, S. aureus, serratia

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16
Q

Diagnose:
- lots of Granuloma formation
- Lymphadenopathy and hepatosplenomegaly

A

Chronic granulomatouus disease

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17
Q

deficiency of NADPH oxidase

A

Chronic granulomatous disease

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18
Q

Dihydrorhodamine flow cytometry test doest change to fluorescent rhodamine with H2O2

A

Chronic granulomatous disease

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19
Q

Inability to form granulomas

A

IL-12, IL12-R, INFg, INFg-R deficiencies

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20
Q

which cells produce IL-12

A

macrophagesWh

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21
Q

which cell responds to IL-12

A

t cells and then produce IFNg

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22
Q

Which cells produce TNF-alpha

A

macrophages

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23
Q

IL-12, IL12-R, INFg, INFg-R deficiencies have a susceptibility to which infections

A

recurrent infections with encapsulated bacteria - NHS - neisseria, Haemophilus, streptococcus

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24
Q

Failure to generate free radicals

A

chronic grnaulomatous disease

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25
Q

PLACESS - catalase positive

A

Pseudomonas
Listeria
Aspergillus
Candida
E. coli
S. aureus
Serratia

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26
Q

organism that infects macrophages

A

mycobacterium

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27
Q

Infections in phagocyte deficiency

A

Bacteria - Staphylococcus aureus, enteric bacteria
Fungi - Candida albicans, Aspergillus fumigatus

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28
Q

Infections in IL-12 deficiency

A

TB, atypical mycobacteria

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29
Q

Absent neutrophil count
Normal leucocyte adhesion markers
No neutrophils for NBT/DHR
No pus

A

Kostmann syndrome

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30
Q

High neutrophil count
Absent CD18
Normal NBT/DHR
No pus

A

Leucocyte adhesion deficiency

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31
Q

Normal neutrophil count
Normal leucocyte adhesion markers
Abnormal NBT/DHR
Pus present

A

Chronic granulomatous disease

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32
Q

Normal neutrophil count
Normal leucocyte adhesion markers
Normal NBT/DHR
Pus present

A

IL12/IFN-gamma deficiency

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33
Q

Treatment for Chronic granulomatous disease

A

IFN-g therapy

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34
Q

absence of NK cells in the peripheral blood
vs
NK cells are present but function is abnormal

A

Classical NK deficiency - absence of NK cells in the peripheral blood
Functional NK deficiency - NK cells are present but function is abnormal

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35
Q

Infections in NK cell deficiency

A

Increased risk of viral infections
(e.g. HSV, CMV, EBV, VZV)

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36
Q

Tx - NK cell deficiency

A

Prophylactic aciclovir
IFN-alpha - stimulate NK cytotoxic function
HSC transplant

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37
Q

Diagnosis: Recurrent infections with NO neutrophils on FBC

A

Kostmann syndrome

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38
Q

Diagnosis: Recurrent infections with HIGH neutrophils on FBC and no pus formation

A

Leucocyte adhesion deficiency

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39
Q

Diagnosis: Recurrent infections with hepatosplenomegaly and abnormal DHR

A

Chronic granulomatous disease

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40
Q

Diangosis: Infection with atypical mycobacteria
Normal FBC

A

IFN-gamma receptor deficiency

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41
Q

Diagnosis: Severe viral infections (e.g. chickenpox, disseminated CMV)

A

Classical NK cell deficiency

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42
Q

Factor H?

A

A regulatory protein in the alternative complment pathway

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43
Q

complement deficiency, common infections

A

Neisseria meningitidis
Haemophilius influenzae
Streptococcus pneumoniae
NOTE: susceptibility to N. meningitidis is particularly common in properidin and C5-9 deficiency

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44
Q

Mannose binding lectin deficiency

A

common but no immunodeficiency

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45
Q

Common terminal pathyway deficiency

A

Defect in C3, C5-C9
inability to make MAC, inability to lyse encapsulated bacteria

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46
Q

Severe meningococcal septicaemia - complement deficiency?

A

C7

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47
Q

Immunodeficiency in membranous glomerulonephritis

A

Secondary C3 deficiency
Nephritic factors stabilize C3 convertases -> C3 activation and consumption.

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48
Q

Cause of secondary C1, C2, C4 deficiency

A

Active lupus

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49
Q

C2 def

A

all have SLE, severe skin disease and high infections

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50
Q

Presents @ 3m with:
- Infections – all types
- FTT
- Persistent diarrhoea
- Unusual skin disease
- Colonisation of infant’s empty bone marrow by maternal lymphocytes à Graft vs host disease

A

SCID

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51
Q

X-linked SCID vs SCID (general)

A

X-Linked SCID has immature B cells, general SCID has no B cells

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52
Q

Mutation in X-linked SCID

A

Mutation of the common gamma chain of
the IL-2 receptor on Ch Xq13.1

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53
Q

adenylate kinase 2(AK2) mutation

A

Adenosine Deaminase def

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54
Q

presentation of Adenosine Deaminase def

A

low B, T and NK cells.

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55
Q

Deletion at Chr22q11.2

A

DiGeorge Syndrome

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56
Q
  • Cardiac abnormalities à TOF
  • Abnormal facies – high forehead, low
    set ears
  • Thymic aplasiaà T cell lymphopenia
  • Cleft palate
  • Hypocalcaemia/hypoparathyroidism
A

DiGeorge Syndrome

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57
Q

Absent expression of MHC class II molecules

A

Bare Lymphocyte Syndrome Type 2
CD4+ t helper cell deficiency - no class switching

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58
Q

Clinically:
- Unwell by 3 months & FTT
- Infection – all types
- May be associated w/ sclerosing
cholangitis
- FHx of sudden infant death.

A

Bare Lymphocyte
Syndrome Type 2

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59
Q

Failure to express CD40L on activated T cells

A

Hyper IgM syndrome

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60
Q

TPMP polymorphism , don’t give

A

azathioprine -> susceptible to bone marrow failure

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61
Q

Cyclophosphamide SE

A

Haemorrhagic cystitis

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62
Q

Cyclophosphamide - MOA

A

Alkylates the guanine base of DNA -> damages DNA and prevents replication

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63
Q

Calcineurin inhibitors

A

Tacrolimus and Cyclosporin

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64
Q

mutation of MCH III

A

Common Variable
Immune Deficiency

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65
Q

JAK1and3 inhibitor

A

Tofacitinib

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66
Q

Defective B cell tyrosine kinase gene (BTK)

A

Bruton’s X-linked hypogammaglobulinemia

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67
Q

Mutation in ChXq26

A

Hyper IgM Syndrome

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68
Q

absence of mature B cells and no
circulating Ig after ~ 3 months

A

Bruton’s X-linked hypogammaglobulinemia

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69
Q

most common primary immunodeficiency

A

IgA deficiency

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70
Q

Renal cell cancer (most commonly used one)

A

IL-2

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71
Q

Chronic granulomatous disease à stimulates phagocytes

A

INF-g

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72
Q

Relapsing MS, Bechet’s

A

INFb

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73
Q

HCV, HBV, Kaposi

A

INFa

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74
Q

hairy cell leukaemia, CML, myeloma

A

INFa

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75
Q

Azathioprine - type of drug

A

Purine analogue

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76
Q

Azathioprine blocks?

A

blocks de novo synthesis of
adenine and guanine

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77
Q

Azathioprine - MOA

A

prevents DNA
replication

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78
Q

Azathioprine - metabolite

A

6-Mercaptopurine

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79
Q

Methotrexate - MOA

A

Inhibits dihydrofolate reductase (DHFR)
Decreases DNA synthesis

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80
Q

JAK 1 and 3 inhibitor indications ?

A

rheumatoid and psoriatic arthritis

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81
Q

Calcineurin inhibitors indications?

A

Rejection prophylaxis in transplantation

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82
Q

CH50 functional complement assay tests what?

A

test of classical pathway (C1, 2, 4, 3, 5-9)

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83
Q

Classical pathway has

A

1, 2, 4, 3, 5-9

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84
Q

AP50 functional complement assay tests what?

A

test of the alternative pathway (B, D, Properidin, C3, C5-9)

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85
Q

Alternative pathway has what?

A

B, D, Properidin, C3, C5-9

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86
Q

Severe childhood-onset SLE with normal levels of C3 and C4

A

C1q deficiency

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87
Q

Membranoproliferative nephritis with abnormal fat distribution (partial lipodystrophy)

A

C3 deficiency with nephritic factor

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88
Q

Meningococcus meningitis with a family history of a sibling dying aged 6

A

C7 deficiency

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89
Q

Recurrent infections when neutropaenic following chemotherapy, but previously well

A

MBL deficiency

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90
Q

Mutation in common gamma chain on Xq13.1

A

X-linked SCID

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91
Q

Main immuno problem in X-linked SCID

A

IL-2 Receptor deficient
This is a component of many cytokine receptors leading to an inability to respond to cytokines,
Causing arrest in T and NK cell development and the production of immature B cells

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92
Q

two mechanisms by which CD8+ T cells kill cells

A

Perforin and granzyme
Fas ligand

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93
Q

immunological consequences of an underdeveloped thymus gland

A

Normal B cell count
Low T cell count
Homeostatic proliferation with age

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94
Q

Normal CD8+
Very low CD4+
Normal B cell count
Low IgG

Unwell by 3 months of age
Infections of all types
Failure to thrive
Family history of early death

A

Bare Lymphocyte syndrome type 2

Deficiency of MHC Class II means that CD4+ T cells cannot be selected in the thymus leading to CD4+ T cell deficiency

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95
Q

CD4 low
CD8 low
B cells normal/low
IgM normal/low
IgG low

A

SCID

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96
Q

CD4 low
CD8 low
B cells normal
IgM normal
IgG normal/low

A

Di George

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97
Q

CD4 low
CD8 normal
B cells normal
IgM normal
IgG low

A

BLS Type 2

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98
Q

severe recurrent infections from 3 months of age, CD4 and CD8 are absent, B cells present, Ig low, normal facial features and echocardiogram

A

X-linked SCID:

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99
Q

young adult with chronic infection with Mycobacterium marinum

A

IFN-gamma receptor deficiency

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100
Q

recurrent infections in childhood, abnormal facial features, congenital heart disease, normal B cells, low T cells, low IgA and IgG

A

22q11.2 deletion syndrome

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101
Q

6-month old baby with two recent serious bacterial infections. T cell present but only CD8. IgM present but IgG is low.

A

Bare lymphocyte syndrome type 2

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102
Q

Class of Ig determined by?

A

Heavy chain

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103
Q

Effector function of Immunoglobulin

A

Fc portion of heavy chain

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104
Q

abnormal B cell tyrosine kinase (BTK) gene

A

Bruton’s X-linked hypogammaglobulinaemia
Prevents the maturation of B cells at that point at which they emerge from the bone marrow

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105
Q

Boys present in the first few years of life
Recurrent bacterial infections (e.g. otitis media, pneumonia)
Viral, fungal and parasitic infections
Failure to thrive

A

Bruton’s X-linked hypogammaglobulinaemia

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106
Q

mutation in the CD40 ligand gene

A

Hyper IgM syndrome

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107
Q

adenylate kinase 2

A

Reticular dysgenesis

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108
Q

Blockage of maturation of IgM cells through germinal centres into B cells that produce other classes of Immunoglobi

A

X-linked Hyper IgM syndrome

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109
Q

Normal B cells
Normal T cells
No germinal centre reactions
High IgM
Absent IgG, IgA and IgE (failure of isotype switching)

A

X-linked Hyper IgM syndrome

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110
Q

Boys present in the first few years of life
Recurrent infections (mainly bacterial)
Subtle abnormality in T cell function (predisposes to PCP, autoimmunity and malignancy)
Failure to thrive

A

X-linked Hyper IgM syndrome

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111
Q

Marked reduction in IgG, IgA and IgE
Poor/absent response to immunisation

A

CVID

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112
Q

Failure of differentiation of B lymphocytes

A

CVID

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113
Q

May present in adults or children
Recurrent bacterial infection (often severe)
Pulmonary disease (e.g. interstitial lung disease)
GI disease (e.g. IBD-like disease)
Autoimmune disease (e.g. AIHA)
Malignancy (e.g. NHL)

A

CVID

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114
Q

most common immunodeficiency

A

IgA deficiency

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115
Q

CD4 low
CD8 low
B cell low
IgM low
IgG low
IgA low

A

SCID

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116
Q

CD4 normal
CD8 normal
B cell low
IgM low
IgG low
IgA low

A

Bruton’s X-linked Hypogammaglobulinaemia

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117
Q

CD4 normal
CD8 normal
B cell normal
IgM high
IgG low
IgA low

A

X-linked Hyper IgM syndrome

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118
Q

CD4 normal
CD8 normal
B cell normal
IgM normal
IgG normal
IgA low

A

Selective IgA deficiency

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119
Q

CD4 normal
CD8 normal
B cell normal
IgM normal
IgG low
IgA low

A

Combined variable immunodeficiency

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120
Q

adult with bronchiectasis, recurrent sinusitis and development of atypical SLE

A

CVID

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121
Q

1-year old boy. Recurrent bacterial infections, CD4 and CD8 cells present, B cells absent, absent IgG, IgA and IgM

A

Bruton’s X-linked hypogammaglobulinaemia

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122
Q

recurrent bacterial infections as a child, episode of PCP, high IgM, absent IgA and IgG

A

X-linked hyper IgM syndrome

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123
Q

recurrent respiratory tract infections, absent IgA, normal IgM and IgG

A

IgA deficiency

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124
Q

driven by components of the innate immune system - disease type?

A

Autoinflammatory = driven by components of the innate immune system

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125
Q

driven by components of the adaptive immune system - disease type?

A

Autoimmune = driven by components of the adaptive immune system

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126
Q

Does autoinflamm or autoimmune have HLA associations?

A

Autoimmune

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127
Q

Does autoinflamm or autoimmune have autoantibodies?

A

Autoimmune

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128
Q

Monogenic autoinflammatory?

A

Familial mediterranean Fever

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129
Q

List examples of polygenic autoinflammatory diseases

A

Crohn’s

UC

Osteoarthritis

Giant cell arteritis

Takayasu’s

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130
Q

monogenic autoimmune diseases

A

IPEX
ALPS

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131
Q

List examples of polygenic autoimmune diseases

A

Rheumatoid arthritis

Myasthenia

Pernicious anaemia

Graves disease

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132
Q

List examples of mixed pattern diseases

A

Ankylosing spondylitis

Psoriatic arthritis

Behcet’s

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133
Q

Monogenic autoinflammatory disease - which cytokine signalling pathways are affected?

A

TNF-alpha or IL-1

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134
Q

Which protein is upregulated in autoinflammatory diseases caused by gain-of-function mutation in NLRP3?

A

Cryopyrin (aka NLRP3)

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135
Q

Mutation of NLRP3 diseases?

A
  • Muckle Wells syndrome
  • Familial cold autoinflammatory syndrome
  • Chronic infantile neurological cutaneous articular syndrome/Neonatal Onset Multisystem inflammatory disorder (NOMID)
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136
Q

Mutation of NLRP3 inheritance pattern

A

AD

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137
Q

Gene mutation in FMF

A

MEFV gene

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138
Q

What does the MEFV gene encode

A

Pyrin-marenostrin:
ordinarily a negative regulator of the inflammatory pathway → mutation leads to increased inflammation

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139
Q

Describe the inflammasome complex function

A

The pathway is activated by toxins, pathogens and urate crystals
These act via cyropyrin and ASC (apoptosis-associated speck-like protein) to activate procaspin 1
Activation of procaspin 1 results in the production of NFkB, IL-1 and apoptosis

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140
Q

Does loss or gain of function of pyrin-marenostrin lead to hyperactivity of the inflammasome complex?

A

loss of function

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141
Q

Does loss or gain of function of cryopyrin lead to hyperactivity of the inflammasome complex?

A

Gain of function

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142
Q

inheritance pattern of Familial Mediterranean Fever

A

AR

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143
Q

Cells that contain Pyrin-marenostrin

A

Neutrophils

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144
Q

Periodic fevers lasting 2-4 days associated with:

Abdominal pain (peritonitis)
Chest pain (pleurisy, pericarditis)
Arthritis
Rash

A

FMF

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145
Q

Complications of FMF

A

AA Amyloidosis -> renal failure

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146
Q

treatment of FMF

A

Colchicine 500 micrograms
- it bind to tubulin and disrupts neutrophil migration and chemokine secretion

Anakinra - IL-1 receptor blocker
Etanercept - TNF-alpha blocker

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147
Q

APECED stands for?

A

Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy

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148
Q

Mechanism of defects in AIRE

A

AIRE = transcription factor - regulates expression of self-antigens

Defect = failure of central tolerance and release of auto-reactive T cells

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149
Q

Autoimmune conditions in APECED

A

Hypoparathyroidism (COMMON)
Addison’s disease (COMMON)

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150
Q

Patients with APECED more prone to what infection and why?

A

Candida - they produce antibodies against IL-17 and IL-22

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151
Q

What does IPEX stand for?

A

Immune dysregulation polyendocrinopathy enteropathy X-linked syndrome

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152
Q

mutation in IPEX

A

FoxP3 - required for dvlpt of Treg cells

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153
Q

“Diabetes, dermatitis, Diarrhoea”

A

IPEX

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154
Q

What does ALPS stand for?

A

Autoimmune lymphoproliferative syndrome

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155
Q

mutation in FAS pathway

A

ALPS

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156
Q

Immune consequence of FAS pathway mutation

A

Mutations in the FAS pathway leading to defects in apoptosis of lymphocytes

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157
Q

High lymphocyte count
Large spleen and lymph nodes
Autoimmune disease (usually cytopaenias)
Lymphoma - over time

A

ALPS

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158
Q

mutation in Crohn’s disease

A

NOD2 - aka CARD-15 on chromosome 16

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159
Q

What is NOD2 - where? what does it recognise?

A

found in cytoplasm of myeloid cells - acts as microbial sensor - recognises MURAMYL DIPEPTIDE

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160
Q

Crohn’s treatment?

A

Corticosteroids
Azathioprine
Anti-TNF-alpha antibodies
Anti-IL 12/23 antibodies

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161
Q

HLA-B27

A

Ank spon

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162
Q

Ank spon heritability

A

90%

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163
Q

Immunosuppressive Tx for ank spon

A

Anti-TNF-alpha
Anti-IL17

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164
Q

HLADR3

A

Graves and SLE

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165
Q

HLADR3/4

A

T1DM

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166
Q

HLADR4

A

Rheumatoid Arthritis

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167
Q

HLADR15

A

GOodpasture’s

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168
Q

frequent mutation in polygenic autoimmune

A

PTPN22
CTLA4

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169
Q

What does PTPN22 do?

A

suppresses T cell activation

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170
Q

What does CTLA4 do?

A

regulates T cell function (expressed by T cells)

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171
Q

Anaphylactic hypersensitivity - immediate hypersensitivity which is IgE-mediated

A

Type I Hypersensitivity

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172
Q

Immune complex hypersensitivity - antibody reacts with soluble antigen to form an immune complex

A

Type III Hypersensitivity

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173
Q

Delayed-type hypersensitivity - T cell mediated response

A

Type IV Hypersensitivity

174
Q

Cytotoxic hypersensitivity - antibody reacts with cellular antigen

A

Type II Hypersensitivity

175
Q

Type I inflammatory pre-formed mediates

A

HIstamine
Serotonin
Proteases

176
Q

Type I inflammatory synthesised mediates

A

Leukotrienes
Prostaglandins
Bradykinin
Cytokines

177
Q

IgE mediated type I response

A

IgE binds to a foreign antigen (e.g. pollen)

The Fc portion binds to mast cells and basophils leading to degranulation

178
Q

Antibodies binding to cellular antigens lead to cell death how?

A

antibodies can activate complement (by binding to C1) or bind to NK cells and macrophages resulting in phagocytosis

179
Q

Autoantigen in Goodpasture’s

A

non-collagenous domain of basement membrane collagen IV

180
Q

Autoantigen in Pemphigus vulgaris

A

Epidermal cadherin

181
Q

Autoantigen in Grave’s disease

A

TSH receptor

182
Q

Autoantigen in Myasthenia gravis

A

Nicotinic acetylcholine receptor

183
Q

Tpye IV hypersensitivity mediated diseases

A

Insulin-dependent DM - pancreatic beta-cell antigen
MS - myelin basic protein, proteolipid protein, myelin oligodendrocyte glycoprotein

184
Q

Graves’ disease antibody

A

Anti TSH receptor

185
Q

Hashimoto’s antibodies

A

Anti-thyroid peroxidase (TPO) antibodies

Anti-thyroglobulin antibodies

186
Q

Gel and Coombs class for T1DM

A

Type IV Hypersensitivity
CD8 T cell mediated destruction of pancreatic islet cells

187
Q

Autoantibodies in T1DM

A

Anti-glutamic acid dehydrogenase (GAD)
Anti-islet antigen (IA2)
Anti-islet cell
Anti-insulin

188
Q

Major complication of B12 def

A

Subacute degeneration of the spinal cord (involving the posterior and lateral columns)
Megaloblastic anaemia

189
Q

myasthenia gravis antibody

A

nicotinic acetylcholine receptors

190
Q

Progessive muscle weakness following repetitive activity
Drooping eyelids, double vision
Dysarthria, dysphasia
Proximal muscle weakness
Symptoms worse at the end of the day

A

Myasthenia gravis

191
Q

Tensilon test

A

Myasthenia gravis
administer very short-acting acetylcholinesterase inhibitor which causes a rapid improvement in symptoms (rarely used)

192
Q

Goodpasture’s syndrome - antibody

A

Anti-GBM

193
Q

What does Anti-GBM bind to?

A

Collagen type IV

194
Q

Lungs - SoB, haemotypsis, widespread crackes
Kidneys - haematuria, oedema, reduced urine output, hypertension

A

Goodpasture’s syndrome

195
Q

RA polymorphisms

A

HLA DR1
HLA DR4
PTPN22
PAD 2 and PAD 4 polymorphisms

196
Q

peptidylarginine deiminases (PAD) (enzyme involved in what?)

A

RA

197
Q

Porphyromonas gingivalis gum infection causes?

A

RA

198
Q

What is a pannus

A

inflamed synovium in RA

199
Q

What diesease are ANA found in

A

Connective tissue autoimmune disease

200
Q

SLE hypersensitivity type?

A

III - antibodies bind to antigens -> immune complexes form and deposit in tissues

201
Q

Two types of ANA are?

A

Anti-dsDNA - specific for SLE
Anti-ENA - extractable nuclear antigens - Scl-70, centromere

202
Q

Complement titres to measure in SLE

A

C3 and C4 will be low

203
Q

immunofluorescence patterns between dsDNA and ENA antigens

A

anti-dsDNA = homogenous
anti-ENA = speckled pattern

204
Q

Livedo reticularis on skin

A

Anti-phospholipid syndrome

205
Q

antibodies in antiphospholipid syndrome

A

Anti-cardiolipin
Anti-beta2 glycoprotein-1
Lupus anticoagulant

206
Q

pathophysiology of Sjorgren’s syndrome?

A

Autoimmune destruction of exocrine glands

207
Q

Sjogren’s antibodies

A

Anti-Ro and Anti-La

208
Q

Sjogren’s malignancy risk of?

A

MALT lymphoma

209
Q

main features of limited cutaneous systemic sclerosis

A

Calcinosis

Raynaud’s phenomenon

Esophageal dysmotility

Sclerodactyly

Telangiectasia

NOTE: also pulmonary hypertension

210
Q

Cytokines involved in Systemic sclerosis

A

Th2 and Th17
Type I collagen alpha 2 chains
FIbrillin 1
TGF-beta

211
Q

antibody seen in limited cutaneous systemic sclerosis

A

anti-centromere

212
Q

antibody seen in diffuse cutaneous systemic sclerosis

A

anti-topoisomerase (aka anti-Scl70)

213
Q

perivascular CD4+ T cell and B cells are seen, this can cause an immune complex-mediated vasculitis (type III response)

A

Dermatomyositis

214
Q

CD8+ T cells surround HLA Class I expressing myofibres, CD8+ T cells kill these myofibres via granzyme/perforin (type IV response)

A

Polymyositis

215
Q

Heliotrope rash
Gottron papules
Photosensitivity

A

Dermatomyositis

216
Q

Gold standard for inflamm myopathies

A

Muscle biopsy

217
Q

anti-aminoacyl tRNA synthetase (e.g. Jo-1), anti-Mi2

A

Dermatomyositis

218
Q

anti-aminoacyl tRNA synthetase antibody is cytoplasmic

A

Polymyositis

219
Q

dsDNA+ve

A

SLE

220
Q

ENA +ve

A

SLE
Sjogren’s
Diffuse Cutaneous Systemic Sclerosis
Limited Cutaneous Systemic Sclerosis

221
Q

Cytoplasmic ANA +ve

A

Myositis

222
Q

Classification system for systemic Vasculitides

A

Chapel Hill

223
Q

Small vessel vasculitides

A

Microscopic polyangiitis (pANCA)
Eosinophililc Granulomatosis with polyangiitis (pANCA)
aka Churg-Strauss syndrome
Granulomatosis with polyangiitis (cANCA)

224
Q

cANCA antiboy

A

Proteinase-3

225
Q

pANCA antibody

A

myeloperoxidase

226
Q

cANCA fluorescence

A

Cytoplasmic fluorescence

227
Q

pANCA staining pattern

A

Perinuclear staining pattern

228
Q

How B cells undergo clonal expansion

A
  1. differntiate into T-cell independent IgM plasma cells
  2. Germinal centre reaction and becoem IgG/A/E memory and plasma cells
229
Q

Does CD8 or CD4 undergo more pronounced proliferation following activation

A

CD8

230
Q

Antigen-presenting cells

A

Dendritic
Macrophages
B lymphocytes

231
Q

Cell surface receptor in influenza vaccine

A

Haemagglutinin (HA)

232
Q

Which receptors on RBCs bind to Haemagglutinin A on influenza virus

A

Sialic acid receptors

233
Q

Influenza protection lasts?

A

6 months

234
Q

Type of BCG vaccine

A

attenuated

235
Q

Mantoux test?

A

Liquid tuberculin (PDD) is injected intradermally

after 48-72 hrs, a reaction would appear as a wheel around the injection site (this is suggestive of latent TB, active TB or previous BCG)

236
Q

What is a live attenuated vaccine? List some examples.

A

MMR,
Varicella zoster,
BCG,
Oral polio (Sabin)
Yellow fever,
nasal influenza, typhoid,

237
Q

adv of live attenuated vaccines

A
  • lifelong immunity
  • activates all phases of immune system
  • broad immune response to multiple antigens
238
Q

disadv of live attenuated vaccines

A

Reversion to virulence:
- Spread to contacts (immunosuppressed patients)
- paralytic poliomyelitis
- storage problems

239
Q

Inactivated vaccines

A

Inlfuenza (quadrivalent)
Cholera
Polio (Salk)
Hep A
Pertussis
Rabies

240
Q

Toxoid vaccines

A

Diphtheria
Tetanus

241
Q

Component/Subunit vaccines

A

Hep B (HBsAg)
HPV (capsid)
Influenza (HA)

242
Q

adv of inactivated/component vaccines

A

no risk of reversion to virulent form
immunodeficient patients can use
storage eaiser
lower cost

243
Q

disadv of inactivated/component vaccines

A

multiple injections
poor immunogenicity

244
Q

conjugate vaccines mechanism?

A

Polysaccharide and protein carrier

245
Q

List some examples of conjugate vaccines.

A

encapsulated bacteria

Haemophilus influenzae type B
Meningococcus
Pneumococcus

246
Q

adjuvants - how they work

A

They mimic the action of PAMPs on TLR and other PRRs

247
Q

examples of adjuvants

A

aluminiumm salts
lipids - monophosphoryl lipid A

248
Q

what is the most common adjuvant

A

aluminium salts

249
Q

protein in SAR-CoV-2

A

spike protein

250
Q

What are dendritic cell vaccines?

A

Used against cancer
DCs collected from patient and exposed to tumour antigen to boost immune response

251
Q

Immunoglobulin prepared from thousands of pooled donors
Covers wide range of unspecified antigens
Contains pre-formed IgG
Administered IV or SC

A

Human normal immunoglobin

252
Q

IL-2 cytokine therapy mechanism

A

stimulates T cells in renal cancer

253
Q

IFN-gamma cytokine therapy mechanism

A

enhance macrophage function in CGD

254
Q

IFN-alpha cytokine therapy mechanism

A

enhance macrophage function in CGD

255
Q

When might specific immunoglobulin be given?

A

post-exposure prophylaxis for:

Varicella Zoster
Tetanus
Hepatitis B
Rabies

256
Q

Boosting immune response, types of adoptive T cell transfer? [4]

A

Virus-specific T cells
Tumour infiltrating T cells (TIL)
T cell receptor T cells (TCR)
Chimeric antigen receptor T cells

257
Q

How does Virus-specific t-cell therapy work?

A

Take WBCs from patient or someone matched -> stimulate in vitro with EBV antigens -> expansion of EBV specific T cells -> put back in patient

258
Q

TCR and CAR T cell therapy

A

Take T cell from patient with malignancy -> insert genes to express [T cell receptor / antibody and T cell receptor] -> expand and put back into pt.

259
Q

Tisagenlecleucel

A

CAR T cell therapy for CD19

260
Q

Tisagenlecleucel targets what?

A

CD19

261
Q

Tisagenlecleucel treats what?

A

NHL and ALL

262
Q

What is the difference between TCR and CAR T cell transfer?

A

TCR is specific for HLA/MHC molecules on tumour cell
CAR T is specific for antigens on the tumour surface

263
Q

Ipilimumab is what?

A

monoclonal antibody

264
Q

Ipilimumab blocks what?

A

CTLA4 on T cells -> blocks inhibition and allows more T cell proliferation

265
Q

Ipilimumab is used when?

A

Advanced melanoma

266
Q

Pembrolizumab and nivolumab are what?

A

monoclonal antibodies

267
Q

Pembrolizumab and nivolumab block what?

A

PD-1 that is expressed on T cells

268
Q

Pebrolizumab and nivolumab MOA

A

Inhibit negative signal from ligands on tumour cells -> by binding PD-1 -> increased T cell activation and proliferation

269
Q

When are Pembrolizumab and nivolumab used?

A

advanced melanoma, NSCLC, metastatic renal cell carcinoma

270
Q

How much prednisolone = endogenous steroid?

A

3-4 mg pred

271
Q

Effect of steroids on prostaglandins?

A

Corticosteroids inhibit phospholipase A2

272
Q

What does phospholipase A2 do?

A

converts phospholipids into arachidonic acid which is subsequently converted into prostaglandins and leukotrienes by cyclo-oxygenases

273
Q

Steroids on macrophages?

A

Decreases macrocyte trafficking to site of inflammation
Decreased endothelial adhesion molecule expression (results in transient neutrophilia)
Decreases phagocytosis
Decreases release of proteolytic enzymes

274
Q

Steroids on lymphocytes?

A

Lymphopenia
Blocks cytokine gene expression
Decreases antibody production
Promotes apoptosis

275
Q

Anti-proliferative agent

A

Cyclophosphamide
Mycophenolate
Azathioprine
Methotrexate

276
Q

Alkylates the guanine base of DNA which inhibits replication

A

Cyclophosphamide

277
Q

Cyclophosphamide used for?

A

SLE
Vasculitis
NHL

278
Q

Cyclophosphamide Side effects?

A

Haemorrhagic cystitis
Bladder cancer
Bonde marrow suppression
Sterility in males

279
Q

What is the most toxic anti-proliferative?

A

cyclophosphamide

280
Q

Blocks de novo purine synthesis by inhibiting HGPRT

A

azathioprine

281
Q

Azathioprine metabolised to?

A

6-mercaptopurine in the liver

282
Q

HGPRT stands for

A

Hypoxanthine-guanine phosphoribosyltransferase

283
Q

Indications for azathioprine

A

Bone marrow suppression
Hepatotoxicity

284
Q

Precaution (polymorphism) with Azathioprine

A

TPMT activity needs checking

TPMT required for azathioprine inactivation and metabolism
- 1 in 300 individuals have TPMT polymorphism which means that they are unable to metabolise azathioprine leading to severe bone marrow suppression

285
Q

TPMT stands for?

A

Thiopurine methyltransferase

286
Q

Dangerous CI with azathioprine

A

Allopurinol

287
Q

Allopurinol inhibits what?

A

Xanthine oxidase

288
Q

Blocking de novo purine nucleotide synthesis by inhibiting IMPDH thus preventing DNA replication

A

Mycophenolate mofetil

289
Q

Indications for mycophenolate mofetil

A

transplant immunosuppression

290
Q

Adverse effects of mycophenolate mefetil

A

BM suppression
Herpes reactivation
Progressive leukoencephalopathy (JC virus)

291
Q

indications for plasmapheresis

A

Goodpasture’s
Severe acute myasthenia gravis
Antibody mediated transplant rejection

292
Q

How do calcineurin inhibitors work?

A

calcineurin blocks IL-2 production

293
Q

Describe the calcineurin pathway

A

Normally, TCR engagement leads to increased cytoplasmic calcium which binds to calmodulin leading to the activation of calcineurin
Calcineurin then activates NFATc resulting in the upregulation of IL-2
IL-2 acts back on T cells to stimulate activation and proliferation

294
Q

Calcineurin inhibitors?

A

Ciclosporin
Tacrolimus

295
Q

INdications for calcineurin inhibitors

A

Transplant
RA
Eczema severe
Psoriasis
Psoriatic arthritis
IBD (UC)

296
Q

JC virus

A

progressive multifocal leukocencephalopathy

297
Q

JC virus associated with use of what?

A

Mycophenolate mofetil

298
Q

Plasmapheresis

A

cell separator removes autoreactive Igs from patient blood

299
Q

rebound antibody production?

A

After plasmapheresis, plasma cells that remain still start producing antibodies, despite antibodies being removed initiallyWh

300
Q

What is often given with plasmapheresis

A

Anti-proliferative agents

301
Q

Ciclosporin risks

A

Nephrotoxic
HTN
Neurotoxic
Dysmorphic features !!

302
Q

Tacrolimus risks

A

Nephrotoxic
HTN
NEurotoxic
Diabetogenic !!

303
Q

What do mTOR inhibitors do?

A

Inhibit T cell activation and proliferation

304
Q

Example of mTOR inhibitor

A

Sirolimus

305
Q

What is Tumour infiltrating T cell transfer?

A

Remove pt tumour -> culture with IL-2 -> expansion of tumour-specific T cells -> put back into patient

306
Q

Advanced melanoma is treated with what immune checkpoint blocking agent?

A

Ipilimumab
OR
Pembrolizumab + Nivolumab

307
Q

What treats relapsing MS?

A

INF-beta

308
Q

What treats CGD?

A

IFN-gamma

309
Q

What treats HCV?

A

IFN-alpha

310
Q

What treats hairy cell leukaemia?

A

IFN-alpha

311
Q

What treats Renal cell carcinoma (Cytokine therapy)

A

IL-2

312
Q

What treats Behcet’s?

A

IFN-beta

313
Q

What treats Kaposi, CML, Myeloma and HBV

A

IFN-alpha

314
Q

Which anti-proliferative agent affects B cells more than T cells?

A

Cyclophosphamide

315
Q

Which anti-proliferative agent affects T cells more than B cells?

A

Mycophenolate mofetil
Azathioprine

316
Q

metabolite of azathioprine

A

6-Mercaptopurine

317
Q

Mechanism of Anti-proliferative / cytotoxic agents

A
  • Damages DNA (cyclophosphamide)
  • Prevents or decreases DNA replication (Mycophenolate mofetil, Azathioprine, Methotrexate)
318
Q

Inhibits dihydropholate reductase

A

Methotrexate

319
Q

Purine analogue that blocks de novo adenine and guanine synthesis

A

Azathioprine

320
Q

Purine anti-metabolite that blocks de novo guanine synthesis

A

Mycophenolate mofetil

321
Q

Inhibitors of cell signalling

A

Tacrolimus
Cyclosporin
Tofacitinib
Apremilast

322
Q

MOA of these inhibitors of cell signalling:
TACROLIMUS
Cyclosporin
Tofacitinib
Apremilast

A

Tacrolimus - Inhibit T cell prolif; blocks IL-2 production

323
Q

MOA of these inhibitors of cell signalling:
Tacrolimus
CYCLOSPORIN
Tofacitinib
Apremilast

A

Cyclosporin - Inhibit T cell prolif; blocks IL-2 production

324
Q

MOA of these inhibitors of cell signalling:
Tacrolimus
Cyclosporin
TOFACTINIB
Apremilast

A

Tofacitinib - reduces JAK STAT singalling

325
Q

MOA of these inhibitors of cell signalling:
Tacrolimus
Cyclosporin
Tofacitinib
APREMILAST

A

Apremilast - Increased cAMP -> increased PKA -> reduced cytokine production

326
Q

JAK 1 & 3 inhibitor example

A

Tofactinib

327
Q

Calcineurin inhibitors examples

A

Tacrolimus
Cyclosporin

328
Q

PDE4 inhibitor?

A

Apremilast

329
Q

Rejection prophylaxis in transplantation?

A

Cyclosporin or Tacrolimus

330
Q

Indication for JAK inhibitor

A

Rheumatoid arthritis
Ulcerative colitis
Psoriatic arthritis
Axial spondyloarthritis

331
Q

Indication for PDE4 inhibitor

A

Psoriasis
Psoriatic Arthritis

332
Q

Basic mechanism:
1. BASILIXIMAB
2. Abatacept
3. Rituximab
4. Vedolizumab
5. Natalizumab

A

anti-CD25 (alpha chain of IL-2 receptor)

333
Q

Basic mechanism:
1. Basiliximab
2. ABATACEPT
3. Rituximab
4. Vedolizumab
5. Natalizumab

A

CLTA4-Ig

334
Q

Basic mechanism:
1. Basiliximab
2. Abatacept
3. RITUXIMAB
4. Vedolizumab
5. Natalizumab

A

anti-CD20

335
Q

Basic mechanism:
1. Basiliximab
2. Abatacept
3. Rituximab
4. VEDOLIZUMAB
5. Natalizumab

A

anti-alpha4beta7 integrin

336
Q

Basic mechanism:
1. Basiliximab
2. Abatacept
3. Rituximab
4. Vedolizumab
5. NATALIZUMAB

A

anti-alpha4beta1 integrin

337
Q

-cept means?

A

Receptor + Immunoglobulin

338
Q

adverse-effects of anti-thymocyte globulin

A

Infusion reactions
Leukopaenia
Infection
Malignancy

339
Q

Basiliximab and Daclizumab MOA

A

anti-CD25 (alpha chain of IL-2 receptor)

340
Q

What interleukin can’t be produced with Basilixima and Daclizumab

A

IL-2

341
Q

Abatercept MOA

A

bind to CTLA4 T cell via CD80 and CD86 receptors

Inhibits CD28 engagement on T cell -> no activation

342
Q

Rituximab MOA

A

Anti-CD20 -> depletion of mature B cells

343
Q

Indications for rituximab

A

B cell Lymphoma
RA
SLE

344
Q

How often Rituximab given?

A

IV 2 doses every 6-12 months

345
Q

Adverse effects of rituximab

A

Progressive multifocal leukoencephalopathy (PML)
CVD exacerb
Infusion Rx

346
Q

Vedolizumab MOA

A

Blocks alpha-4-beta-7-integrin -> inhibits leukocyte migration

347
Q

Vedolizumab use?

A

IBD

348
Q

Vedolizumab adverse effects?

A

Infusion Rx
Hepatotoxic
PML
Malignancy

349
Q

Tocilizumab / Sarlimumab MOA

A

Antibody against IL-6

350
Q

Indication for Tocilizumab / sarlimumab

A

RA
Giant Cell Arteritis
Castleman’s disease - IL6 producing tumour from HHV8

351
Q

Anti-TNF-alpha antibodies

A

Infliximab
Adalimumab
Certolizumab
Golimumab

352
Q

How are Anti-TNF-alpha Ab given?

A

Subcut
Infliximab can also be IV

353
Q

Anti- TNF-alpha indications?

A

IBD
Ank Spon
RA
Psoriasis and Psoriatic arthritis
FMF

354
Q

side-effects of anti-TNFα antibodies

A

Infusion reactions
Infection (TB reactivation, HBV, HCV)
Lupus-like conditions
Demyelination
Malignancy

!!! screen for latent TB before starting

355
Q

etanercept MOA

A

TNFalpha/TNFbeta receptor p75-IgG fusion protein -> receptor goes round binding to TNF

356
Q

Ustekinumab MOA

A

Anti IL-12 and anti IL-23

357
Q

Secukinumab MOA

A

Anti-IL-17a

358
Q

Guselkumab MOA

A

Anti-IL23 -> ab against p19 subunit

359
Q

Denosumab MOA

A

Anti-RANK ligand -> inhibits RANK mediated osteoclast differentiation

360
Q

Osteoporosis Immune med

A

Denosumab

361
Q

IBD Immune meds

A

Infliximab (/adalimumab / certolizumab / golimumab)
Ustekinuma

362
Q

Throat swelling wihtouth decreased BP?

A

C1 esterase inhibitor deficiency

363
Q

What conditions is the IL-23 and IL-17 pathway important for?

A

Ankylosing spondylitis, psoriasis and psoriatic arthritis, IBD (not IL-17)

364
Q

Which antibodies inhibit IL-17 and IL-23?

A

Secukinumab - IL-17
Guselkumab - IL-23

365
Q

Interleukins in Asthma and Eczema

A

IL4
IL5
IL13
Th2 and eosinophil response

366
Q

Antibodies treating asthma and Eczema?

A

Dupilumab - IL-4
Mepolizumab - IL-5
Tralokinumab - IL-13

367
Q

Types of infusion reaction

A

IgE mediated (T1 hypersensitivity)
Non-T1 Hypersensitivity

368
Q

Urticaria
Hypotension
Tachycardia
Wheeze
After infusion

A

IgE-mediated infusion reaction

369
Q

Headache
Fever
Myalgia
After infusion

A

Non-T1 hypersensitivity infusion reaction

370
Q

injection site reactions peak when?

A

48 hrs

371
Q

Injection site reaction -> clinical sign?

A

Cutaneous necrosis

372
Q

Diseases to screen for before immunosuppression

A

TB, HBV, HCV, HIV

373
Q

JC virus important in immunosuppression why?

A

John Cunningham Virus
Common polyomavirus - majority of population has been exposed to it
Usually kept well under control by immune system
In the case of severe immunosuppression, the virus can reactivate and destory oligodendrocytes, leading to PML

374
Q

immunosuppression agents leading to PML (JC virus activation)?

A

Vedolizumab
Rituximab

375
Q

Reaction type in allergic disorder

A

IgE mediated Type I hypersensitivity

376
Q

Th2 response?

A
  1. Damaged epithelium releases signalling molecules
  2. cytokines act on Th2, Th9 and ILC2 cells
  3. They then produce IL4, IL5 and IL13
  4. These act on basophils and eosinophils
  5. IL4 stimulates B cells to produce IgE and IgG4
377
Q

Allergens can cross-link IgE leading to mast cell degranulation and the release of histamines, prostaglandins and leukotrienes

A

Allergic response initiated by parasites and allergens
Not mediated by Th2 cells

378
Q

Peptide presentation via MHC to TCR or Th2 cells

Induces production of what IL?

A

IL-4

379
Q

How does oral route promote tolerance to allergense?

A

Treg in GI mucosa inhibits IgE synthesis -> promotes tolerance

380
Q

Theories behind increasing Allergies

A
  1. Hygiene hypothesis - reduced childhood infection increases allergy susceptability
  2. Lack of vitamin D and fatty acids in diet
  3. Loss of symbosis with gut and respiratory bacteria due to altered composition and biodiversity
  4. Increase in epithelium-damaging agents due to industrialisation
381
Q

Functional allergen tests?

A

Mast cell tryptase
Basophil activation test
Allergen challenge

382
Q

Allergen specific IgE (sensitisation) tests?

A

Skin Prick
Serum IgE (RAST)

383
Q

Ix collected in acute episode

A

Serial Mast cell tryptase
Blood/urine histamine

384
Q

Pos skin prick/specific IgE Test = ?

A

Sensitisation not allergy

385
Q

Specific IgE Test prognostic markers?

A

Concentration - higher levels means more symptoms
Affinity to the target - higher affinity means increased risk
Capacity of IgE antibody to induce mast cell degranulation

386
Q

Biomarker for anaphylaxis

A

Mast cell tryptase

387
Q

How is mast cell released during anaphylaxis

A

Tryptase is pre-formed protein found in mast cell granules
Systemic degranulation during anaphylaxis results in increased serum tryptase

388
Q

Mast cell tryptase peaks?

A

1-2 hours peak
6-12 hrs baseline

389
Q

MMast cell tryptase doesnt return to baseline?

A

Systemic mastocytosis

390
Q

Gold standard for food/drug allergy?

A

Challenge test

391
Q

Basophils increase which markers when activated

A

CD64
CD203
CD300

392
Q

emergency management of anaphylaxis in adults

A

0.5mL of 1:1000 (>12 years old)

give 2 doses if no improvement after first
If still no improvement -> refractory anaphylaxis

393
Q

common drug cause of angioedema

A

ACEi

394
Q

Cytokine key for dvlpt of eosinophils

A

IL-5

395
Q

most common organ transplant

A

Kidney then liver

396
Q

Half life of transplanted kidney

A

12 yrs

397
Q

HLA encoded on which chromosome?

A

Chromosome 6

398
Q

Alleles of HLA class I

A

A, B, C

399
Q

Alleles of HLA Class 2 (2 letters for class 2)

A

Class II - DR, DQ, DP

400
Q

Basic structure of HLA Class I

A

Domains:
3 alpha
1 beta-2 microglobulin
1 transmembrane

401
Q

Basic structure of HLA Class II

A

2 alpha
2 beta
2 transmembrane

402
Q

HLA Class I where?

A

All cells

403
Q

HLA Class II where?

A

APC

404
Q

HLA alleles most immunogenic

A

A, B, DR

405
Q

HLA mismatch code e.g. 1:1:0 are for?

A

A, B, DR Alleles

406
Q

Gold-standard diangosis of graft rejection

A

biopsy

407
Q

Types of rejection

A
  1. T-cell mediated
  2. Antibody-mediated
408
Q

T-cell mediated rejection MOA

A

1) presentation of donor HLA by APC in context of recipient HLA
2) T cell activation, inflamm cell recruitment
3) Organ damage - effector phase

409
Q

Features of T-cell mediated rejection

A

Lymphocytic interstitial infiltration
Tubulitis - WBCs in tubular epithelium
Arteritis - WBCs within vascular wall

410
Q

3 signals of T cell activation

A

Antigen/MHC interaction with TCR
Co-stim molecular expression - CD80/96 interaction with CD28
IL-2

411
Q

AB antigens present where?

A

Endothelial cells
Erythrocytes

412
Q

Antibody mediated rejection phases?

A

1) B cells recognise foreign HLA
2) Prolif of B cells and anti-HLA antibody production
3) Effector phase - Ab bind to graft endothelium -> intravascular disease

413
Q

Anti-AB vs Anti-HLA antibodies?

A

Anti-AB antibodies are naturally occuring (pre-formed)
Anti-HLA antibodies are not naturally occuring but can be pre-formed due to previous exposure to epitopes

414
Q

Presence of inflammatory cells within the capillaries of the graft - hallmark for?

A

Antibody-mediated transplant rejection

415
Q

Assays for anti-HLA antibodies

A

Cytotoxic assays
Flow cytometry
Solid phase assays

416
Q

Targets for T-cell pathway immunosuppression in transplants

A

Calcineurin inhibitors
IL-2 inhibitors - e.g. Anti-CD25 antibody = Basiliximab

417
Q

Drugs that target TCR

A

Muromonab - anti-CD3 antibody
Anti-thymocyte globulin

418
Q

Anti-CD52 antibody

A

Alemtuzumab

419
Q

Anti-CD20 antibody

A

Rituximab - depletes B-cells

420
Q

BAFF inhibitor - BAFF required for B cell survival

A

Belimumab

421
Q

Proteosome inhibitor

A

Bortezomib - inhibits degradation of pro-apoptosis proteins

422
Q

Complement inhibitor example?

A

Eculizumab - Targets C5

423
Q

Graft-vs-Host-Disease Pathophys?

A

During SCT, the host immune system is eradicated and replaced by autologous or allogeneic bone marrow
In allogeneic stem cell transplant, donor lymphocytes can attack host tissues

424
Q

Preventing GvHD

A

Methotrexate + ciclosporin

425
Q

Opportunistic infections in Transplant recipients

A

CMV
BK virus
PCP

426
Q

Malignancies in transplant recipients

A

Kaposi sarcoma
EBV
Skin cancer

427
Q

Define positive tryptase test

A

> 1.2 baseline + 2

428
Q

Milky fluid produced by rubber trees (Hevea brasiliensis)

A

Latex

429
Q

Complement deficiency investigations

A

CH50
AP50
C3 and C4

430
Q

Main interferon in SLE

A

Type 1 INF

DEFNE Extra Buzzwords (14 decks)

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