Immuno

Question 1

Atypical granulomatous infection in previously healthy patient

Answer 1

IFN gamma, IFNgamma receptor deficiency, IL-12, IL-12 receptor
Salmonella, atypical
Mycobacteria, TB and BCG.

Patients are unable to form granulomata

Question 2

6 days every 3 weeks; give G-CSF

Answer 2

Cyclic neutropenia

Question 3

X linked tyrosine kinase - BTK gene

Failed production of mature B cells, no antibodies, symptoms 3-6 months

Answer 3

Brutons agammaglobulinaemia

Question 4

congenital neutropenia=NO NEUTROPHILS

Answer 4

Kostmann

Question 5

Rheumatoid arthritis patient with mycobacterial infections

Answer 5

Iatrogenic - TNF antagonists

Question 6

Recurrent infections with negative NBT and dihidrorhodamine tests

Answer 6

Chronic granulomatous disease

Question 7

Young girl with normal B cells, normal CD8+ but absence of CD4+ (jaundice and hepatosplenomegaly). kid unwell by 3 months of age:

Answer 7

Bare Lymphocyte Syndrome BLS. IgG would be low

(Answer type 1 and 2 exist, but more common to have type 2 with absent CD4 compared to type 1 with absent CD8).

Question 8

Young boy with normal B cells and absence of CD8+ and CD4+

Answer 8

Di George

Question 9

Immune deficiency that has improved with age but not mum is concerned about delayed speaking and language

Answer 9

Di George

Question 10

Normal B and T cells, high IgM but absence of IgA, IgE and IgG– Pneumocystis Cariini and failure to thrive.

Answer 10

Answer – CD40L deficiency – this is hyper-IgM. Presents in first years of life with recurrent bacterial infections. X-linked; can’t class switch so no IgA and IgG

Question 11

Patient with recurrent pneumococcal infections and meningitisforming membrane attack complex) can predispose to infection from encapsulated organisms.

Answer 11

C5-C9 Deficiency

Question 12

A 6-month old boy with severe failure to thrive and recurrent infections, early infant death…etc… his T cells were undetectable and B cells were NORMAL even if you think they should be lower

Answer 12

(Answer – X-linked SCID) Failure to thrive, and deficiency in IL-2.

Question 13

SLE. Membraneous lymphogromelulonephritis

Answer 13

C1q deficieny.

Question 14

Marker of antibody mediated rejection

Answer 14

C4d

Question 15

Most important to match for rejection

Answer 15

HLA: DR>B>A

Question 16

Hyper-acute rejection (ABO incompatibility)

Answer 16

Preformed Ab activates complement

Thrombosis and necrosis, prevented by cross matching properly

Question 17

Can lead to development of post transplantation lymphoproliferative disease. PTLD is a proliferation (rapid increase) of lymphoid (immune) cells. It can develop in people who are taking immunosuppressive drugs to prevent rejection of an organ or an allogeneic (donor) bone marrow or stem cell transplant

Answer 17

calceurin inhibitors

Question 18

Causes progressive multifocal leukoencephalopathy

Answer 18

mycophenolaTe myofetil T cells

Question 19

Mechanism behind GVHD Graft versus host disease (GvHD)

Answer 19

○ Donor cells attacking host
○ Days-weeks, rash, N+V, bloody diarrhoea, abdo pain, jaundice
○ Prophylaxis: methotrexate/cyclosporine
○ Treat with steroids

Question 20

Mechanism behind antibody mediated rejection

Answer 20

○ Antibodies attack vessels
○ Exposure -> proliferation + maturation of B cells ->
○ Effector phase - antibodies bind to graft endothelium
(->vasculitis), two weeks after the actual transplant

Question 21

Mechanism behind cellular rejection

Answer 21

○ T cells, type IV reaction
○ TReat with T cell suppressors
○ Donor APC presenting antibodies to recipient
○ Cellular infiltrate

Question 22

Diffuse systemic scleroderma

Answer 22

scl-70 topoisomerase

Question 23

Limited cutaneous scleroderma

Answer 23

anti-centromere

Question 24

Anti phospholipid syndrome

Answer 24

anti-cardiolipin

Question 25

epistaxis and haematuria

Answer 25

Wegener’s granulomatosis c-ANCA

Question 26

Serum sickness - Type X reaction?

Answer 26

III reaction, following antibiotics, symptoms take 7-12h to develop

Question 27

dry eyes and mouth antibodies

Answer 27

anti-Ro, anti-La

Question 28

weight loss, anti TTG antibodies, anti-endomysial antibodies, steatorrhoea

Answer 28

Coeliac

Question 29

CREST

Answer 29

Calcinosis, Raynaud’s, Esophageal dysmotility, Sclerodactyly, Telangiectasia

Question 30

SLE

Answer 30

anti ds DNA

Question 31

Pernicious anaemia

Answer 31

anti parietal cell > anti intrinsic factor

Question 32

heamoptysis and haematuria

Answer 32

goodpasture, anti-collagen membrane IV

Question 33

How to monitor the activity of SLE?

Answer 33

C3 & C4 levels

Question 34

How to monitor a patient with HIV?

Answer 34

CD4 T lymphocyte cell count to assess immune function and plasma HIV RNA (viral load) to assess level of HIV viremia.

Question 35

How to monitor Autoimmune Hepatitis?

Answer 35

Anti-SM

Question 36

FAS pathway

Answer 36

REMEMBER FAS IN THE ALPS: ALPS syndrome, auto-immune lymph-proliferative syndrome, mutation in the FAS gene which steers lymphoproliferation of the immunce cells. ALPS is characterized by the production of an abnormally large number of lymphocytes (lymphoproliferation). Accumulation of excess lymphocytes results in enlargement of the lymph nodes (lymphadenopathy), the liver (hepatomegaly), and the spleen (splenomegaly). FAS drives high lymphocyte proliferation, thus BIG BIG ALPES of hepatmogelay, SPLENOMGALY, SPLENOMEGALY

Question 37

Mentioned MEFV gene, periodic abdominal pain and ascites.

Answer 37

Familial Mediterranean Fever

Question 38

abnormal production of IL-1 and the attack of the pleura surfaces

Answer 38

Familial Mediterranean Fever

Question 39

Nod2/CARD15

Answer 39

Crohns Disease

Question 40

Single gene mutation involving FOXp3 resulting in the abnormality of Treg cells (Which normally regulate T-helper cell function)

Answer 40

IPEX – (Immune dysregulation polyendocrinopathy enteropathy an X-lined) is caused by

Question 41

Autoimmune Polyendocrinopathy Syndrome Type 1 - AIRE mutation.

Answer 41

``` 2/3 components: (regulatory trush) chronic mucocutaneous candidiasis, hypoparathyroidism adrenocortical insufficiency Treg cell defect. ```

Question 42

Hereditary angioedema. A patient with some description [can’t remember details] of C1 inhibitor deficiency – she was still able to talk

Answer 42

C1 inhibitor deficiency C1 inhibitors, which are responsible for maintaining proper balance (homeostasis) in the complement system (specifically, keeping the C1 part of the classical complement pathway from being overactive). C1q esterase needed

Question 43

When they eat cherries, apples, pears and hazelnuts, they get itchy mouths

Answer 43

Acute Angiodema/ Oral Allergy Syndrome

Question 44

Hypertensive and diabetic, with angioedema

Answer 44

ACE inhibitors

Question 45

Woman with flushed face, problems breathing that has happened multiple times with enlarged liver

Answer 45

carcinoid

Question 46

A patient with some rash etc… after eating.

Answer 46

H1 oral antihistamines

Question 47

A patient with some rash and wheeze etc

Answer 47

IM adrenaline

Question 48

Cytokine responsible for promoting antibody synthesis

Answer 48

IgE

Question 49

Main cellular source of histamine

Answer 49

Mast cell

Question 50

How do you measure mast cell degranulation

Answer 50

Mast cell tryptase levels

Question 51

Chemokine responsible for promoting eosinophil growth

Answer 51

IL5 | Secrete Igs, cytokines, present antigens

Question 52

Present antigens; migrate to lymph nodes when activated

Answer 52

Dendritic

Question 53

CD8+

Answer 53

Cytotoxic - MHC I

Question 54

In its immature form it is specialized to phagocyte pathogens, and in its mature form it is specialized to present to other cells

Answer 54

Monocyte (macrophages)

Question 55

Monocytes resident in peripheral skin cells

Answer 55

Langerhans cell

Question 56

Connective Tissue

Answer 56

Histiocyte

Question 57

Cells that express Foxp3 and CD25

Answer 57

Treg

Question 58

Differentiated B cells that produce immunoglobulins

Answer 58

The effect or cells

Question 59

Responsible for the killing of cancerous cells and are inhibited by MHC-I –

Answer 59

NK cells

Question 60

The multimetric immune modulator

Answer 60

ISCOM

Question 61

Can be used for RA depletes B-cells

Answer 61

Anti-CD20 Rituximab,

Question 62

Antibody against IL6 receptor

Answer 62

Used in caslteman’s, RA if not responding to methotrexate, sulfasalazine

Question 63

Pooled Ig used in the treatment

Answer 63

CVID

Question 64

Prophylaxis of allograft rejection, IV before + after Antibody against

Answer 64

IL-2Ralpha chain of CD25 Basiliximab

Question 65

Inhibits T-cell migration and may only be used against highly active remitting/relapsing MS

Answer 65

Anti Alpha-4 integrin Natulizumab

Question 66

Treatment options include; inhibition of IL-12/23, TNF-alpha and IL17a

Answer 66

Psoriasis

Question 67

Treatment options include; inhibition of IL-6, TNF-alpha and depletion of B-cells

Answer 67

Rheumatoid arthritis

Question 68

Antiproliferative agent - B>T cells affected) Alkylates guanine base of DNA, Damages

Answer 68

Cyclophosphamide

Question 69

IFNgamma

Answer 69

Chronic granulomatous disease

Question 70

Used to treat malignant melanoma, involved in T cell checkpoints

Answer 70

ANTI-PD1

Question 71

Used to prevent transplant rejection, by blocking T cell proliferation and activation

Answer 71

mycophenolate myofetil

Question 72

Vaccine that eradicated small pox

Answer 72

Vaccinia

Question 73

Antibody to p40 subunit of IL12 and IL23

Answer 73

Ustekinumab

Question 74

Antiproliferative agent - B>T cells affected

Answer 74

Cyclophosphamide

Question 75

Used to treat malignant melanoma, involved in T cell checkpoints

Answer 75

Pemrolizumab (ANTI-PD1)

Question 76

Hepatitis C, Hepatitis B, Kaposi’s sarcoma

Answer 76

Interferon alpha:

Question 77

Interferon beta

Answer 77

Relapsing MS

Question 78

influenza A only, targets M2 channel

Answer 78

Amantadine

Question 79

NA inhibitors

Answer 79

Oseltamivir, zanamivir, sialic acid