Allergies and the immune system

Question 1

What are the features of anaphylaxis

Answer 1

angiodema
urticarial rash
vomiting
respiratory distress

Question 2

What investigation could be done if you are unsure of an allergic reaction

Answer 2

mast cell tryptase

Question 3

what is hyper-IgE-syndrome

Answer 3

• Autosomal dominant
• rare multisystem primary immunodeficiency disorder
• presentation is in infancy/early childhood

Question 4

How deso hyper-IgE syndrome present

Answer 4

• repeated bacterial infections of the skin and lungs (staph aureus)
• first symptoms often eczema
• skeletal abnormalaties
• facial characteristics
• faltering growth
• deep seated liver abcess
• recurrent/persistent sinusitis and otitis media

Question 5

What mutation is often involved in hyper-IgE syndrome

Answer 5

mutations in the STAT3 gene

Question 6

What abnormal blood results would you expect to see in someone with hyper-IgE syndrome

Answer 6

raised total immunoglobulin E

raised eosinophils

Question 7

What are the features of Wiskot-Aldrich syndrome

Answer 7

1. eczema
2. thrombocytopaenia (bruising/bleeding/petichaie)
3. immunodeficiency - increased risk of infections and opportunistic infection

Question 8

What array of immunoglobulins would you expect to see in Wiskot-Aldrick syndromw

Answer 8

LOW IgM and IgG
RAISED IgE and IgA
poor antibody reaction to vaccines
small platlets with a low platelet mean

Question 9

What is Wiskott Aldrich syndrome

Answer 9

• immunological deficiency and reduced ability to form blood clots
• X linked
• mutation in the WAS gene

Question 10

What is the management of Wiskott Aldrich syndrome

Answer 10

bone marrow transplant

Question 11

What is a type 1 immune reaction

Answer 11

IgE mediated - anaphylaxis, asthma, hayfever, eczema, some food allergies

Question 12

What is a type 2 immune reaction

Answer 12

cyto-toxic antibody dependent - autoimmune haemoytic anaemia, goodpastures syndrome, rheumatic heart disease

Question 13

What is a type 3 immune reaction

Answer 13

immune complex mediated - lupus, serum sickness, post strep, glomerulonephritis

Question 14

What is a type 4 immune reaction

Answer 14

cell mediated - contact dermatitis, mantoux test, some intestinal food allergymm

Question 15

key features of anaphylaxis

Answer 15

• acute onset
• involvement skin/mucosa
• resp compromise
• reduced BP/evidene of end organ dysfunction