11: Transplants, Immunodeficiency, Amyloid

Question 1

Hematopoietic SC transplant

Answer 1

Therapy for hematologic malig -> destroys BM -> need SC transplant -> brand new immune system

Question 2

Which recurrent bacterial infections occur in innate immunodeficiencies with leukocyte dysfunction (LAD, Chediak-Higashi, CGD)

Answer 2

Catalase negative bacteria: strep, enterococcus (ex: staph aureus)

Question 3

Which cells are CD40 and CD40L on?

Answer 3

CD40: B cells
CD40L: T cells

Question 4

Most common significant primary immunodeficiency

Answer 4

CVID

Question 5

Possible first indication of IgA deficiency

Answer 5

Transfusion-related anaphylaxis - transfusion of RBCs may induce anaphylaxis

Question 6

Situations that can induce secondary immunodeficiency

Answer 6

1. Cancer
2. Malnutrition
3. Metabolic disease
4. Chronic illness
5. Treatment-related (chemo, radiation, immunosuppression)

Question 7

AIDs definition

Answer 7

The manifestation of HIV when it affects the body to a degree where immune dysfunction causes 1) opportunistic infections 2) secondary neoplasms 3) neurologic manifestations

Question 8

Ways HIV can spread

Answer 8

1. Anal sex (and other modes of sex)
2. IVDU
3. Blood transfusions
4. Mother to baby

Question 9

Why is anal receptive intercourse most risky for HIV transmission?

Answer 9

Mucosa is very thin (vaginal is also pretty thin)

Question 10

Risk of non-HIV STDs on HIV transmission

Answer 10

Other STDs can cause inflammation and ulceration which can make it easier for HIV to enter bloodstream

Question 11

Three ways HIV can be passed from mother to child

Answer 11

1. Through placenta
2. Through birth canal
3. Through breast milk

Question 12

Antigenic stimulation causing release of NF-kB in HIV: what its supposed to do vs what happens

Answer 12

What its supposed to do: upregulate T cell response

What happens: initiates viral transcription through LTR

Question 13

HIV infection window period

Answer 13

There is a window of no viral detection for about 10 days after HIV exposure

Question 14

How is donated blood tested for HIV? Why does the window period affect this?

Answer 14

Tested with NAT, but that still leaves a 7-14 day window period between exposure and positive NAT where HIV could go undetected in the blood

Question 15

Viral set point for HIV

Answer 15

End of initial viremic spike that might predict prognosis of total CD4 cell loss

Question 16

Clinical latency in HIV

Answer 16

Period of a silent massacre of CD4 cells that may go on for years without sx

Question 17

Clinical steps when an HIV dx is made early in 2021

Answer 17

1. HAART initiated (highly active anti-retroviral therapy)
2. CD4 count monitored
3. Disease may progress but is typically well controlled
4. Prophylactic tx against infections

Question 18

Two non-T cells that get infected with HIV and explain

Answer 18

1. Macrophages: reservoir of HIV in certain tissues

2. Microglia: allows HIV to access CNS -> neuronal damage

Question 19

Amyloidosis: explain the concept

Answer 19

1. Protein synthesis is typically highly maintained
2. In cases of incorrect processing, Misfolding occurs -> Beta pleated sheets
3. If misfolded proteins aren’t broken down -> accumulate -> amyloid

Question 20

Three different things to consider when looking at the types of amyloid

Answer 20

1. Localized or systemic?
2. Acquired or hereditary?
3. Different chemical properties of the amyloid

Question 21

Localized vs systemic amyloid: what can cause these?

Answer 21

Local: local disease, tumor
Systemic: primary or secondary inflammatory states

Question 22

What do AA and AL stand for in amyloid?

Answer 22

AA: amyloid associated
AL: amyloid light chain dz