3 BL Deficiency of adaptive immunity Flashcards Preview

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Flashcards in 3 BL Deficiency of adaptive immunity Deck (17):
1

Locations of abnormalities of development in:
SCID

block in development of lymphoid stem cell -> results in lymphopenia of both T and B cells.

2

Locations of abnormalities of development in:
X-linked (Bruton) Agammaglobinemia

block is between pre-B and B cells, and results in low numbers of B cells (normal T cells).

- block is a defective bruton tyrosine kinase

3

Locations of abnormalities of development in:
X-Linked HyperIgM Syndrome

Defect in Tfh's CD40 or in B cell CD40 →defect in IgM switching to IgG

CD40: surface marker that induces the switch

4

Locations of abnormalities of development in:
Common Variable Immunodeficiency (CVID)

normal #s of pre-B and B cells but:

B cells cannot become plasma cells → cannot make specific antibody

5

Locations of abnormalities of development in:
DiGeorge syndrome

large (45 gene) deletion on chromosome 22→
Abnormal development of 3rd and 4th pharyngeal pouches →
abnormal stroma →
cannot support thymic lymphoid development →
pt will have absent T cells.

6

Which abnormal lymphocyte development is related to tetralogy of fallot?

DiGeorge

7

infections you would expect in a pure B cell deficiency

"high grade"
(extracellular, and pyogenic) infections from bacterial pathogens such as S. aureus, H. influenzae, and S. pneumoniae.

8

infections you would expect in a pure T cell deficiency

severe infections from intracellular pathogens such as viruses, certain bacteria, yeasts and fungi (esp. Candida albicans & Pneumocystis jirovecii).

9

clinical features of DiGeorge syndrome

○ Hypertelorism (eyes are far apart; wide-space)
○ Down-slanting eyes
○ Fish mouth deformity
○ Micrognathia (undersized jaw)
○ Low set ears
○ Can be born with Tetralogy of Fallot, hypocalcemia, and absent T cells.

10

incidence of selective IgA deficiency
- associated syndrome?

• Most common immunodeficiency disease with frequency of about 1 in 500.

○ 10-15x more frequent in those with celiac disease

11

immunological problem of the Nude mouse
- name the human immunodeficiency condition it resembles.

results in absence of thymic stroma (and hair) → no T cells.

Immunologically similar to DiGeorge.

12

enzyme which is absent in some cases of SCID.

adenosine deaminase is absent → accumulation of adenosine in cells severely impairing lymphocyte development → lymphopenia of both T and B cells.

13

Possible approaches to replacing adenosine deaminase in people with SCID

Replace the enzyme:
transfusions of irradiated red cells can be helpful

(adenosine deaminase is abundant in blood)

14

Discuss transplantation therapy in DiGeorge.
complication?

○ Fetal thymus or cultured thymic stromal cells have been used to try to minimize graph vs host disease;

better diagnosis of DiGeorge would aid in the selection of appropriate cases.

15

Discuss transplantation therapy in SCID
complication?

○ BETTER to transplant purified stem cells rather than bone marrow.
-Bone marrow transplantation has had about 50% success rate, but graph vs host disease...
- Prefer sibling donors with Class II MHC match and at least one Class I MHC match.

16

Intravenous Immunoglobulin (IVIG)

What is it? When would you use it?

Ig so clean/good that you can inject right in without reaction

Used when B cell function is deficient.

17

Viruses and/or viral diseases which are immunosuppressive in humans

malaria
measles
mononucleosis (mono)
cytomegalovirus (CMV)
EBV