Immunology Flashcards by Nadja Auerbach

What is bare lymphocyte syndrome?

Defect with MHC Class II expression; NO CD4+, failure to make IgA or IgG antibody

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Acute cellular mediated rejection; what cells are involved and what kind of mechanism does Tx focus on?

T cells, type IV reaction

Treat with T cell suppressors

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Translocation in Mantle Cell Lymphoma

t(11,14)

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Target of immune system in TTP

glycoprotein IIb and IIIa

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Kostmann syndrome, what and how is it inherited?

Autosomal recessive, severe neutropenia

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T cell inhibitor and calcineurin inhibitor

Tacrolimus

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How might Hyper IgM syndrome present? How is it inherited?

Boys present with failure to thrive in first few years of life, recurrent bacterial infections, e.g. PCP.

Normal number of circulating B cells. X-linked

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C3 deficiency presents how?

severe susceptibility to bacterial infections, esp. encapsulated (meningococcus, streptococcus, haemophilus)

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How might CVID present?

recurrent bacterial infections with end organ damage, autoimmune disease, BRONCHIECTASIS

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Type I hypersensitivity plus example?

IgE mediated

e.g. anaphylaxis, atopic dermatitis, oral allergy syndrome

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Gum hyperplasia plus calcineurin inhibitor?

Cyclosporin A

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DHR flow cytometry test; used for what and what result?

Chronic granulomatous disease; would also be negative

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Rituximab MOA and uses?

Anti CD20; RA and lymphoma

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Abatacept

CTL4 immunoglobin fusion protein, Tx in RA

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Drug to treat psoriasis and MOA

Ustekinumab (anti IL12 and 23)

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single gene mutation in FOXp3

IPEX - immune dysregulation poly endo enteropathy x linked

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Cells that express Foxp3 and CD25

T regulatory cells

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subset of cells that express CD4 and secrete IFN gamma and IL2

Th1

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Low IgM, high IgA and IgE

Wiskott Aldrich

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Mouse antibody

OKT3

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Targets neuraminidase/haemogglutinin

Oseltamivir, zanamivir

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Type II hypersensitivity, example?

IgG or IgM mediated

Autoimmune haemolytic anaemia, Goodpasture’s, Grave’s pernicious anaemia, vasculitis conditions

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Anti-topoisomerase

Diffuse scleroderma

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ALPS autoimmune lymphoproliferative syndrome

failure of t cell death, mutations in Fas pathway

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Anti-centromere

Limited scleroderma: CREST

DiGeorge syndrome, what is it and what features?

22q11. deletion ``` CATCH: Cardiac abnormalities Abnormal facies Thymic aplasia (no T cells) Cleft palate Hypocalcaemia ``` Normal number of B cells and reduced T cells

Tx for malignant melanoma and MOA

Ipilimumab ; targets CTLA4 | Pembroluzimab

Patient with recurrent pneumococcal infections and meningitis

complement deficiency

Failure to express CD40L on activated T cells

Hyper IgM syndrome

Autoimmune polyendocrine syndrome

AIRE gene problem

Causes progressive multifocal leukoencephalopathy

John Cunningham virus

What is used to prevent organ transplant rejection? MOA?

Tacrolimus; involved with T cell check points

How does SCID present?

unwell by 3 months of age, persistent diarrhoea, unusual skin disease

Prophylaxis of allograft rejection, IV before + after

Basiliximab

Mycophenate mofetil

Prevent guanine synthesis, used to prevent rejection in allograft

What receptors do phagocytes express?

Toll like receptors | Fc receptors

Chemokine responsible for promoting eosinophil growth

IL5

Type IV hypersensitivity, example?

Delayed, T cell mediated T1DM, MS, RA, Crohn's

Anti-mitochondrial

PBC

Bruton's, what is it and what is the mode of inheritance?

Low B cells, no Ig. | X linked

Recurrent TB infection

IFN-y receptor deficiency

Young girl with normal B cells, normal CD8+ but absence of CD4+

Bare lymphocyte

Myeloperoxidase deficiency

Positive nitro blue test, risk of candida

How to treat methotrexate resistant RA?

Anti-TNF alpha

Type III hypersensitivity, example?

IgG or IgM immune complexes deposited in tissues SLE, Goodpasture's

Defective B cell tyrosine kinase gene

Bruton's X-linked gammaglobinaemia

Patient gets recurrent infections with negative NBT and dihdrorrhadamine tests

Chronic granulomatous disease

Adult with bronchiestasis, recurrent sinusitis and development of atypical SLE. Diagnosis?

Common variable deficiency

Anti-TNF alpha drugs

Infiliximab Etanercerpt Adalimumab

Rituximab MOA

CD20

What organisms does a complement deficiency put you at risk of?

encapsulated bacteria

Translocation in Burkitt's

t(8:14)

Low or absent T cells, normal or high B cells | Poorly developed lymphoid tissue in thymus

X-linked SCID

Vaccine every 5 years given to splenectomy patients

Pneumococcal

HLA association in Rheumatoid?

HLA DR3 and DR4

Leukocyte adhesion deficiency

High number of neutrophils in blood, unable to exit blood. No pus produced

Translocation in follicular lymphoma

t(14,18)

Low IgG, IgA and IgE

Common variable immune deficiency

t(14,18)

follicular lymphoma

Diabetes, eczema, enteropathy

IPEX

First cytokine released when exposed to allergen

IL 12

Levels of CH50 in RA

High; indicator of acute inflammation

Fully humanised TNF-alpha monoclonal Ab

Adalimumab

Stiff man syndrome

Anti GAD

SLE is a deficiency of what part of the complement?

Classical pathway; C1, 2, 4

Ibrutinib can be used in which leukemia?

CLL

Worsening Crohn's, already on Azathioprine and Prednisolone, what tx?

Infliximab - Anti TNF alpha

Inhibits function of lymphoid and myeloid cells and used in management of rheumatoid arthritis

Tocilizumab

Defect in gamma chain of IL-2 receptor

X-linked SCID

Anti CD25 for prophylaxis of allograft rejection, inhibits T cell proliferation

Basiliximab

Abatacept and use?

Anti - CTLA4 Remember: In 'R'heumatoid 'A'rthri'T'is you use 'R'ituximab 'A'batacept and 'Tocolizumab'

Basiliximab and use?

Anti CD25, IL-2 receptor Allograft rejection prophylaxis

Rituximab and use?

Anti CD20 RA Remember: In 'R'heumatoid 'A'rthri'T'is you use 'R'ituximab 'A'batacept and 'Tocolizumab'

Natalizumab and use?

Anti-alpha 4 integrin MS, Crohn's Remember: Natalie the ALPHA-4 female. She is a woman and more women get MS

Tocolizumab and use?

Anti IL-6 RA Remember: In 'R'heumatoid 'A'rthri'T'is you use 'R'ituximab 'A'batacept and 'Tocolizumab'

Tx for severe Type 2 hypersensitivity disease?

Plasmapheresis

anti-TNF alpha drugs

Infliximab ('nf' like TNF) Adalimumab Etanercerpt (intercepts or inter'cerpts' the TNF)

What does HAART consist of?

2 NRTIs + PI (or NNRTI) e.g. Emtricitabine, tenofovir and Efavirenz (NNRTI)

Protein target in MS?

synovial membrane proteins

type of rejection (time frame) in ABO rejection

hyper acute

Which factor causes fibrosis in systemic sclerosis/crest?

TGF-beta

Anti tyrosine phosphatase

Anti-IA-2 antibodies and anti-phogrin in DM

anti liver kidney antibodies

autoimmune hepatitis type 2

Atypical granulmous in preveiously health

IFN-gamma receptor deficiency

Nod2/CARD15

Crohn's

Treatment for MRSA if person has penicillin allergy?

Macrolide, e.g. erythromycin