1. Quiz 3 review | September 21, 2016

2. Quiz 3 Review Autoimmune diseases Immunodeficiencies Transfusion
Transplantation

3. Quiz 3 Review
Autoimmune diseases

4. Lupus Things You Must Know
Typical patient: young woman with butterfly rash
Symptoms unpredictable (relapsing/remitting)
Multisystem (skin, kidneys, joints, heart)
Antinuclear antibodies

5. Lupus: butterfly rash

6. Rheumatoid Arthritis Things You Must Know
Symmetric, mostly small-joint arthritis
Systemic symptoms (skin, heart, vessels, lungs)
Rheumatoid factor
Cytokines (especially TNF) cause damage

7. Rheumatoid arthritis joint deformities

8. Sjögren Syndrome Things You Must Know
Inflammatory disease of salivary and lacrimal glands
Dry eyes, dry mouth
T cells react against some Ag in gland; gland gets destroyed
Increased risk of lymphoma

9. Sjögren syndrome: salivary gland enlargement

10. Scleroderma (Systemic Sclerosis)
Things You Must Know
Excessive fibrosis throughout body: skin, viscera
Claw hands, mask-like face
Microvascular disease also present
Diffuse and limited types

11. Systemic sclerosis: claw hands

12. Quiz 3 Review
Autoimmune diseases
Immunodeficiencies

13. Immune Diseases Outline
Transmission
Defect
Clinical stuff
XLA
X-linked
No mature B cells; no Ig
Infant with recurrent bacterial infections
CVID
non x-linked
Defective Ab production
Infections, AI diseases, lymphoma
IgA deficiency
↓ IgA
Most common. Asx. AI diseases
Hyper-IgM
↓ IgG, A, E
T cell defect
Bacterial and fungal infections
DiGeorge
No thymus
Viral, fungal infections
SCID
half are x-linked
Many B and T cell defects
Bacterial and other infections

14. Quiz 3 Review
Autoimmune diseases
Immunodeficiencies
Transfusion

15. Genotype
Antigens
Blood type AA A AO BB B BO AB
A and B OO
None O

16. Genotype
Antigens
Blood type DD D
Rh + Dd dd
none
Rh -

17. What can go wrong? Transfusion reactions hemolytic non-hemolytic
Other complications
infections
iron overload

18. Quiz 3 Review Autoimmune diseases Immunodeficiencies Transfusion
Transplantation

19. HLA genes are inherited as sets (haplotypes)♂ ♀
parents
four possible haplotype combinations in children

20. HLA genes are polymorphic♂ ♀
A B C DR DQ DP
1 7 w
Dad’s HLA genes
2 8 w
3 44 w
Mom’s HLA genes
11 35 w

21. HLA genes are codominantly expressed

22. time after transplant, months
100
number of mismatches
Class I
Class II
1 or 2
3 or 4
graft survival, % 50 3 6 12
time after transplant, months