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Flashcards in Immunology Deck (280)
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1

What are the 4 hallmarks of immune deficiency?

SPUR
Serious infections
Persistent infections
Unusual infections
Recurrent infections

2

what defines a serious infection?

unresponsive to oral antibiotics

3

what defines persistent infections?

early structural damage
chronic infections

4

what defines unusual infections?

unusual organisms
unusual sites

5

what defines recurrent infections?

two major or one major + recurrent minor infections in one year

6

what 6 features apart from SPURS may be surggestive of primary immune deficiency?

weight loss/ failure to thrive
severe skin rash
chronic diarrhoea
mouth ulceration
unusual autoimmune disease
family history

7

what 2 important infections lead to secondary immune deficiency?

HIV
measels

8

what 3 important treatment interventions lead to secondary immune deficiency?

immunosuppressive therapy
anti-cancer agents
corticosteriods

9

what type of malignancies can lead to secondary immune deficiency?

cancer of the immune system (lymphoma, myeloma, leukemia)
metastatic tumours

10

what biochemical/nutritional disorders can lead to secondary immune deficiency?

malnutrition
diabetes (Type 1 and 2)
dialysis

11

what is a major feature of phagocyte deficiencies?

recurrent infections
(which can affect either common or unusual sites)

12

what are the types of organisms typically infect patients with phagocyte deficiencies?

common bacteria (eg Staph sureus)
unusual bacteria (eg Burkholderia cepacia)
Mycobateria (TB and atypicals)
Fungi (candida, aspergillus)

13

what is the general life cycle of a phagocyte? (ie neutrophil

1. mobilisation of phagocytes and precursos from bone marrow or within tissues
2. upregulation of endothelial adhesion markers causing neutrophil adhesion and migration into tissues
3. phagocytosis and killing of organism
4. activation of other components of the immune system

14

what are the 2 reasons that can cause the failure of neutrophil production?
(and therefore neutropaenia and phacogyte deficiency)

failure of stem cells to differentiate along myeloid lineage
specific failure of neutrophil maturation

15

what is reticular dysgensis?

the failure of stem cells to differentiate along myeloid and lymphoid lineages. megakaryocyte-erythroid lineage is unaffected. most severe form of severe combined immunodeficiency (SCID)

16

when can the secondary defect which causes the failure of stem cells to differentiate along the myeloid lineage occur?

after stem cell transplantation

17

what are the 2 congenital causes of specific failure of neutrophil maturation? (and therefore cause neutropaenia)

Kostmanns syndrome
Cyclic neutropaenia

18

what is Kostmanns syndrome?

rare autosomal recessive disorder causing the specific failure of neutrophil maturation- leads to severe chronic neutropaenia

19

what is Cyclic Neutropaenia?

episodic neutropaenia every 4-6 weeks caused by the specific failure of neutrophil maturation

20

when do patients with Kostmanns syndrome usually present with recurrent infections?

within 2 weeks after birth

21

what is the supportive treatment of Kostmanns syndrome?

prophylactic antibiotics
prophylactic antifungals

22

what is the definitive treatment of Kostmanss syndrome?

stem cell transplantation
Granulocyte colony stimulating factor (G-CSF)

23

what is granulocyte colony stimulating factor (G-CSF)?

a growth factor which assists maturation of neutrophils

24

what rare, primary immunodeficiency is caused by the genetic defect in leukocyte integrins?

leukocyte adhesion deficiency

25

what does leukocyte adhesion deficiency result in?

failure of neutrophil adhesion and migration

26

what is the general clinical picture of leukocyte adhesion deficiency?

marked leukocytosis but with localised bacterial infections that are difficult to detect

27

what 2 receptors do phagocytes possess that bind to opsinons?

Fc receptors (for antibody Fc)
Complement receptor 1 (CR1- for binding to complement which are bound to the antigen)

28

what 2 defect can cause decreased efficiency of opsonisation? (and therefore a phagocyte deficiency)

defect of complement/antibody production
defect in opsonin receptor

29

what is chronic granulomatous disease?

a phagocyte deficiency due to failure of oxidative killing mechanisms

30

in chronic granulomatous disease what causes the failure of oxidative killing mechanisms?

inability to generate oxygen free radicals and so impaired killing of intracellular organisms