Immunology Flashcards

1
Q

What are the pathogenic antibodies in bullous pemphigoid?

A

Anti-Hemidesmosome

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2
Q

A 34 year old man has severe Crohn’s disease. His gastroenterologist wishes to start a biologic drug, infliximab. What infection should be ruled out before commencing the drug?

A

Tuberculosis

Monoclonal antibodies directed against TNF-alpha are associated with activation and dissemination of latent TB. TNF-alpha is vital for the control of TB infection in the lungs. If it is blocked, TB can grow unchecked. Hence, prior to starting treatment with an anti-TNF-alpha antibody, a mantoux test should be performed to exclude exposure to TB

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3
Q

What type of vaccine is comprised of a polysaccharide bound to a immunogenic toxin?

A

Conjugate

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4
Q

What antigen is injected intradermally as part of the mantoux screening test for tuberculosis?

A

Tuberculin Purified Protein Derivative

The size of induration is measured after 48-72 hours (not erythema)

> 5mm is positive in HIV or immunocompromised, > 10mm in IVDU or medium risk populations (including healthcare workers), > 155mm is positive in low risk populations

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5
Q

What is the target of the antibody P-ANCA?

A

Myeloperoxidase

= UC, Eosinophilic Granulomatosis with Polyangiitis, Primary Sclerosing Cholangitis or Microscopic Polyangiitis

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6
Q

What is the target of the antibody c-ANCA

A

Proteinase-3
= Granulomatosis with polynagitis

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7
Q

A 80 year old lady received 2 units of FFP 4 hours ago due to a severe nosebleed and an INR of 4.8 on admission.

She takes wafarin for a metallic heart valve. Over the last hour, she is beginning to feel short of breath, especially when lying down. She is hypotensive at 95/40 and SpO2 of 94% on 8L/min O2.

She is pyrexic at 37.8C.

What complication of blood transfusion is she suffering from?

A

Transfusion Related Acute Lung Injury

Transfusion related acute lung injury (TRALI) is due to the presence of leukocyte antibodies causing white blood cells to aggregate in the pulmonary circulation as it passes through in the blood. The disease is characterised by acute onset pulmonary oedema, dyspnoea, severe hypoxaemia and hypotension. Treatment is usually supportive with a good resolution within 2 days. However, severe TRALI may lead to acute respiratory distress syndrome.

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8
Q

A 21 year old woman reports weight loss, tiredness, diarrhoea and non-bloody offensive smelling stools. She has had three chest infections requiring antibiotics in the last three years. Blood tests are ordered and reveals a hypochromic, microcytic anaemia with low ferritin. Anti-TTG and anti-endomysial antibodies are negative. There are normal levels of IgG, IgM and IgE. No IgA is found.

What is the most likely cause of her gastrointestinal symptoms?

A

Particular caution should be noted in the diagnosis of coeliac disease, as the common autoantibodies that are used for diagnosis are of the type IgA (Anti-Endomysial and Anti-Tissue Transglutaminase).

Hence, in IgA deficiency (1 in 600 people), they may be falsely negative.

IgA levels should always be assessed at the same time when assessing for coeliac disease.

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9
Q

What hematological cells create a “respiratory burst” in order to kill phagocytosed pathogens?

A

Neutrophils

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10
Q

What immunomodulatory agent may be given to treat chronic granulomatous disease?

A

Interferon Gamma

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11
Q

What monoclonal antibody targets IL-17 and is involved in the treatment of psoriasis, psoriatic arthritis and ankylosing spondylitis?

A

Secukinumab

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12
Q

What is the main metabolite of azathiroprine?

A

6-Mercaptopurine

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13
Q

A patient is investigated for a primary immunodeficiency. Lymphocyte counts are normal. However, IgG and IgA are low. There is an excess of IgM. What condition is this patient likely to have?

A

Hyper IgM Syndrome (defective CD40L)

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14
Q

What is the inheritance pattern of reticular dysgenesis?

A

Autosomal recessive

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15
Q

A 34 year old woman was given a blood transfusion yesterday due to low haemoglobin. She is pyrexic with a fever of 38c and feels slightly under the weather. All other observations normal. Infusion site is unremarkable. What is the first line treatment of this complication of transplant?

A

Paracetamol

Febrile non-haemolytic transfusion reaction is the most common reaction to packed red cell blood products.

It is thought to be caused by white blood cells releasing cytokines such as IL-1 during storage.

Symptoms include a fever >38 in mild cases or a high fever >39 with chills and rigors in severe reactions.

Mild reactions are not a reason to stop treatment. It may be appropriate to slow the rate of transfusion, if time allows.

Paracetamol or other NSAIDs may provide symptomatic relief.

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16
Q

A 40 year old woman with Hashimoto’s Disease visits the GP for routine review of medication.

She reported feeling particularly tired recently and visited a private rheumatologist on the advice of friends.

The private rheumatologist ran an autoimmune panel of blood tests showing that she is positive for ANA, anti-TPO antibody positive and positive for rheumatoid factor. Other bloods were unremarkable except for low T3 and T4.

What condition is responsible for her symptoms?

A

Hashimoto’s thyroiditis

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17
Q

A 40 year old woman with Hashimoto’s Disease visits the GP for routine review of medication.

She reported feeling particularly tired recently and visited a private rheumatologist on the advice of friends.

The private rheumatologist ran an autoimmune panel of blood tests showing that she is positive for ANA, anti-TPO antibody positive and positive for rheumatoid factor. Other bloods were unremarkable except for low T3 and T4.

What condition is responsible for her symptoms?

A

Hashimoto’s thyroiditis

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18
Q

The paediatric SpR is called to review a 16 day old infant who is pyrexic and appears unwell. His umbillical cord stump is still present, non-purulent, red, tender and hot. He has a fever of 39C. Blood tests reveal a neutrophil count of 16 (normal range: 1.5-8.0) What primary immunodeficiency is this patient likely to have?

A

Leukocyte adhesion defieiency

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19
Q

What immune cell releases granzyme and perforin when activated?

A

T killer cells

CD8 is a co-receptor for the T cell receptor and recognises MHC class 1.

MHC class 1 is found on most nucleated cells and presents random intracellular peptides to CD8+ T cells.

If a T cell recognises a peptide-MHC class 1 complex, then the T cell activates and kills the target cell.

Please note: Simply writing “CD8” is not enough detail for the mark. CD8 is a cell surface protein. We use cell surface markers to classify T cells. Hence, CD8+ T Cells is acceptable - but CD8+ is not.

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20
Q

What lymphocyte lineage does cyclosporine predominantly inhibit?

A

T lymphocytes

Its a CALCINEURIN INHIBITOR

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21
Q

A 20 year old man is screened for primary immunodeficiencies by his GP after a number of recent, severe infections.

A full blood count was normal.

IgA Normal
IgM Low
IgG Low
What immunodeficiency is this patient likely to have?

A

Common Variable Immunodeficiency

Common Variable Immunodeficiency (CVID) is a diagnosis of exclusion in patients greater than 4 years old. The diagnostic criteria are:

Decrease in serum IgG and a decrease in one of IgM or IgA.
There is a lack of antibody response to antigens or immunisation
More than 4 years old.
Patients will have increased infections with bacteria such as Haemophilus, Strep (usually immunized against) and Staph. There is an increased rate of autoimmune conditions and malignancies. Treatment is with normal human IVIg for life.

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22
Q

An 9 year old boy has had recurrent chest infections and influenza like illnesses since joining primary school.

Blood tests reveal a low lymphocyte count, with very low B cells but normal T cell levels.

There as very low levels of IgM, IgA and IgG.

What immunodeficiency is this patient likely to have?

A

Bruton’s Agammaglobulinaemia

Bruton’s Agammaglobulinaemia is a rare X linked immunodeficiency caused by a mutation in the BTK gene, which codes for a tyrosine kinase required for B cell development. Patients with this condition suffer from recurrent infections, typically from Streptococcal or Haemophilus spp. It often does not manifest until after 6 months, when the child loses passive immunity from the mother when breastfeeding. There is a notable lack of lymphoid tissue (lack of tonsils and adenoids) or lack of lymphadenopathy during infections. There is a decrease in all immunoglobulins, though small amounts may be present. It can usually be managed by injections of normal human IVIg for life.

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23
Q

What assay is used as the first line test for HIV infection?

A

Enzyme Linked Immunosorbent Assay (ELIZA)

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24
Q

What antiretroviral drug is given as IV monotherapy during labour to prevent vertical transmission of HIV-1?

A

Zidovudine

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25
Q

What antigen does the immune system erroneously target in autoimmune thrombocytopenic purpura?

A

Glycoprotein IIb/IIIa

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26
Q

An prenatal autoimmune panel of bloods reveals positive anti-Ro antibody in the blood of a pregnant woman with SLE. What cardiological condition is her unborn baby at risk of?

A

Congenital Heart Block

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27
Q

What serine protease released by neutrophils causes hepatitis and emphysema in patients deficient in alpha-1 antitrypsin?

A

Neutrophil elastase

Neutrophil elastase is a serine protease released by neutrophils and can cause hepatitis and emphysema in patients deficient in alpha-1 antitrypsin, which is an inhibitor of neutrophil elastase.

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28
Q

What viral enzyme is predominantly responsible for the high mutation rate of HIV and other RNA viruses?

A

Reverse Transcriptase

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29
Q

How may mast cell degranulation be measured?

A

Tryptase

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30
Q

In skin prick testing, what is used as a positive control?

A

Histamine

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31
Q

What antiretroviral drug is given as IV monotherapy during labour to prevent vertical transmission of HIV-1?

A

Zidovudine

others:

NRTI: Zidovudine, Abacavir

Non-nucleoside reverse transcriptase inhibitor: Efavirenz

Protease inhibitor: Ritonavir

Integrase inhibitor: Dolutegravir

32
Q

What serine protease released by neutrophils causes hepatitis and emphysema in patients deficient in alpha-1 antitrypsin?

A

Neutrophil elastase

33
Q

Which cytokine is important in the pathogenesis of rheumatoid arthritis, anyklosing spondylitis and inflammatory bowel disease?

A

Tumour Necrosis Factor Alpha

34
Q

What is the specific antigen recognised by the immune system in Goodpasture’s syndrome?

A

Type IV collagen

35
Q

What autoantibody is associated with Granulomatosis with Polyangiitis (Wegner’s Granulomatosis)?

A

Cytoplasmic Anti Neutrophil Cytoplasmic Antibody

36
Q

How long before skin prick testing must antihistamines be stopped to allow for accurate interpretation?

A

48 hours

37
Q

A neutrophil releases preformed myeloperoxidase, defensins and neutrophil elastase. What is this process known as?

A

Degranulation

38
Q

What lymphocyte lineage does azathioprine predominantly inhibit?

A

T lymphocytes

39
Q

Which joints in the hand are classically not affected by rheumatoid arthritis?

A

Distal Interphalangeal Joints

40
Q

What is the maximum number of viral capsids that a molecule of IgM may bind to?

A

10

41
Q

The paediatric SpR is called to review a 21 day old infant who is pyrexic and appears unwell. The parents think he has a problem with his hearing as he has been tugging at his ear. There is pain, tenderness and swelling over the mastoid process of his left ear. Blood tests reveal a neutrophil count of 0.1 (normal range: 1.5-8.0) What primary immunodeficiency is this patient likely to have?

A

Kostmann Syndrome

42
Q

A 80 year old woman was given a blood transfusion yesterday due to low haemoglobin. All observations normal. Infusion site is unremarkable. She feels like she has a fever. What complication of blood transfusion is this patient suffering from?

A

Febrile non-haemolytic transfusion reaction

43
Q

What test is the ability of a patient’s serum to lyse sheep erythrocytes coated with rabbit anti-sheep antibodies?

A

CH50

44
Q

What mouse monoclonal antibody targets CD3 on the surface of T cells?

A

Muromonab

45
Q

Mutation in what cell surface receptor may confer immunity from HIV?

A

CCR5- means virus cannot enter the cell to replicate

46
Q

Mutation in what cell surface receptor may confer immunity from HIV?

A

CCR5- means virus cannot enter the cell to replicate

47
Q

What is the term given to proteins, typically produced by the liver in response to infection, that include IL-1, ferritin, IL-6 and CRP.

A

Acute Phase Proteins

48
Q

Ritonavir is an example of what class of antiretroviral drug?

A

Protease inhibitor

49
Q

What enzyme, deficient or defective in chronic granulomatous disease, is responsible for the “respiratory burst” that may kill phagocytosed pathogens?

A

NADPH oxidase

50
Q

What clinical criteria are used for the diagnosis of acute rheumatic fever?

A

Jones Criteria

51
Q

What monoclonal antibody targets alpha4 integrin and is used in the treatment of relapsing remitting multiple sclerosis?

A

Natalizumab

52
Q

What is the long term management of Common Variable Immunodeficiency?

A

Normal Human Immunoglobulin

Common Variable Immunodeficiency (CVID) is a diagnosis of exclusion in patients greater than 4 years old. The diagnostic criteria are:

Decrease in serum IgG and a decrease in one of IgM or IgA.
There is a lack of antibody response to antigens or immunisation
More than 4 years old.
Patients will have increased infections with bacteria such as Haemophilus, Strep (usually immunized against) and Staph. There is an increased rate of autoimmune conditions and malignancies. Treatment is with normal human IVIg for life.

53
Q

Which subtype of T helper cells are implicated in the development of allergic diseases, such as asthma or eczema

A

T helper 2 cells

54
Q

What is the descriptor given to a solution of antibodies that recognise multiple antigens?

A

Polyclonal

55
Q

What is the primary target of the antibody C-ANCA?

A

c-ANCA = Proteinase-3 = Granulomatosis with polyangiitis (Wegner’s)

p-ANCA = Myeloperoxidase = UC, Eosinophilic Granulomatosis with Polyangiitis, Primary Sclerosing Cholangitis or Microscopic Polyangiitis

56
Q

What is the gold-standard test for diagnosis of food allergy?

A

Double Blind Oral Food Challenge

57
Q

What lymphocyte lineage does mycophenolate mofetil predominantly inhibit?

A

T lymphocytes

58
Q

A 31 year old accountant reports an itchy rash over the trunk and back that has been present for the last 7 weeks. On examination, there are smooth erythematous papules with evidence of excoriation. She reports no association with food, time and has changed her washing powder to a “Non-Bio” formulation.

What condition is she suffering from?

A

Chronic Urticaria

59
Q

What is the most common inherited primary immunodeficiency?

A

Selective IgA Deficiency

60
Q

Immunoglobulins may bind to bacteria to allow phagocytosis to occur. What is this process known as?

A

Opsonisation

61
Q

Zidovudine is an example of what class of antiretroviral agents?

A

Nucleoside Reverse Transcriptase Inhibitor

62
Q

An autoimmune panel of bloods reveals positive anti-Jo-1 antibody in a woman with breast cancer.

What autoimmune condition is associated with this autoantibody?

A

Dermatomyositis

63
Q

What monoclonal antibody targets the IL-6 receptor and is involved in the treatment of rheumatoid arthritis?

A

Tocilizumab

64
Q

An autoimmune panel of bloods reveals a positive anti-mitochondrial antibody. What autoimmune condition is associated with this autoantibody?

A

Primary Biliary Cirrhosis

65
Q

What is the name given to a substance that increases the effectiveness of an immune reponse to a vaccination without altering the specificity of the response?

A

Adjuvant

66
Q

Immunoglobulins may bind to multiple pathogens at once in order to enhance phagocytosis.

What is this process known as?

A

Agglutination

67
Q

Mastocytosis suggests an infection by what class of organism?

A

Parasites

68
Q

A 34 year old man has severe Crohn’s disease. His gastroenterologist wishes to start an anti-TNF drug to see if it controls his symptoms better. What first-line test should the patient undergo before starting adalimumab?

A

Mantoux test- anti TNF-Alpha is associated with activation and dissemiation of latent TB

69
Q

Immunoglobulins may bind to cell surface receptors on viruses, to prevent viral binding and entry of human cells.

What is this function of immunoglobins known as?

A

Neutralisation

70
Q

What is the name given to the immunological process that must have occurred prior to a hyperacute transplant rejection?

A

Sensitisation

71
Q

An alcoholic patient presents to their GP for the first time following years of excessive drinking and poor self care.

The GP undertakes a number of investigations, including a blood film.

What neutrophil abnormality would you expect to see?

A

Hypersegmented Neutrophils- MICROCYTIC ANAEMIA

72
Q

An alcoholic patient presents to their GP for the first time following years of excessive drinking and poor self care.

The GP undertakes a number of investigations, including a blood film.

What neutrophil abnormality would you expect to see?

A

Hypersegmented Neutrophils- MICROCYTIC ANAEMIA

73
Q

A severe asthmatic presents to their GP for the third time in three years complaining of blocked sinuses. However, over the last 6 months, she has also had unintentional weight loss and reports intermittent fevers. She also has an indistinct erythematous macular rash on both her ankles.

A Full Blood Count reveals an eosinophilia (>10% of white blood cells).

What rare immunological condition may be responsible for her symptoms?

A

Eosinophilic Granulomatosis with Polyangiitis

74
Q

Which type of immunoglobulin is primarily secreted into breast milk and is responsible for passive immunity in newborns?

A

Immunoglobulin A

75
Q

A 13 year old girl is diagnosed with type 1 diabetes mellitus following hospital admission for diabetic ketoacidosis.

Her thyroid function and liver function tests are normal.

What other autoimmune disease should she be screened for on this admission?

A

Coeliac Disease

Overlapping HLA- DR3