IHO - Immune problems Flashcards Preview

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Flashcards in IHO - Immune problems Deck (9):
1

X linked (bruton's) agammaglobulinemia

X linked
Defect in BTK (tyrosine kinase)
No B cell Maturation -> lack of Immunoglobin
present 6 months

2

Selective IgA Deficiency

Most common primary immune def
Sinupulmonary/mucosal pathogens
IgM, IgG normal
False + HcG

3

Common variable immune def (CVID)

defective B and helper T (bacteria, enterovirus, giardia)
Low plasma cell -> low Ig
Lymphoma risk

4

Digeorge syndrome

T cell deficiency - lack of thymus
3rd and 4th pharyngeal pouch failure 22q11 deletion
- hypo calc as well

5

Severe Combined Immunodef (SCID) (3 kinds)

fungal/viarl/bacterial/protozoa

-Cytokine receptor defects- lack of prolif of T and B cells

-Adenosine deaminase deficiency - toxic build up of adenosine and deoxyadenosine

- MHC Class II deficiency - CD4 need

6

Hyper IgM Syndrome

-elevated IgM
-Mutated CD40L on helper T cells or CD 40 on receptor B cells
- Low IgA, IgG, IgE -> pyrogenic infections

7

Wiskott Aldrich Syndrome
Triad

Triad
-Thrombocytopenia, eczema, recurrent infection
Mutation in WASP gene - X linked

Low IgM; High IgE and IgA

8

Complement Deficiency

C5-C9 -> increased Neisseria risk

9

C1 inhibitor deficiency

loss of control
hereditary angiodema -
-especially periorbital and mucosal surfaces