Immuno/Path Dzs

Question 1

Pt with. C1 esterase deficiency. PD will find? Mech?

Answer 1

Hereditary angioedema. Increased kalkerin activation - increased bradykinin - increased vasodilation

Question 2

Pt with recurrent pyogenic sinus and respiratory tract infections. Increased susceptibility to type III hypersensitivity reactions?

Answer 2

C3 deficiency

Question 3

Recurrent Neisseria infections - immune defect?

Answer 3

C5-C9 (no mac complex)

Question 4

With with occasional hemolobinuria. Possible immune defect?

Answer 4

Paroxysmal nocturnal Hb-uria. Deficiency in DAF

Question 5

Pt with fever, urticaria, arthralgias, and proteinuria a week after exposure to drug?

Answer 5

Serum sickness. HSR III

Question 6

Intredermal injection of vaccine. Get edema, necrosis and complement activation in that area?

Answer 6

Arthus reaction

Question 7

Pt given blood. Gets urticaria, wheezing and fever? tx?

Answer 7

Type I HSR. Give antihistamines

Question 8

PT given blood. Has trouble breathing, hypotension and goes into shock.

Answer 8

Anaplylaxis. IgA deficient pt got blood with IgA

Question 9

Pt given blood.Gets fever, headaches, chills and flushing

Answer 9

HSR II (host Abs against donor HLAs)

Question 10

Pt given blood. fever, hypotension, increased HR, increased RR, flank pain and jaundice

Answer 10

HSR II. Intravascular hemolysis (wrong blood group)

Question 11

Pt with recurrent bacterial infections after 6 months of age. Defect?

Answer 11

X-linked Bruton’s agammaglobuliemia.

BTK gene, no B-cell maturation

Question 12

Pt with recurrent sinusitis and pulmonary infections and numerous allergies. Also has milk allergies and diarrhea.

Answer 12

Selective IgA deficiency. (decreased IgA leads to respiratory infections and GI infections)

Question 13

young adult patient with numberous sinopulmonary infections. Has Lupus. Normal B cells but low plasma cells? Risk for?

Answer 13

Common variable immunodefiency. Lymphoma

Question 14

Pt with small lymph nodes, decreased B-cells and low IgGs. Defect?

Answer 14

X-linked Bruton’s agammaglobuliemia.

BTK gene, no B-cell maturation

Question 15

Female pt with recurrent sinusitis and pulmonary infections and numerous allergies. Has positive B-HCG but not pregnant. Cause?

Answer 15

Selective IgA deficiency.

Question 16

Pt with tetany and recurrent viral/fungal infections. No thymic shadow. Dx?

Answer 16

DeGeorges

Question 17

Pt with recurrent dessemminated infections. Labs show?

Answer 17

decreased INF-y. IL-12 receptor deficiency (few Th1s)

Question 18

Pt with course facial features, rash and non-inflammed abcesses.

Answer 18

Hyper-IgE (Jobs).

Question 19

Hyper IgE - findings?

Answer 19

FATED
course Facies
noninflammed staph Abcesses
retained primary Teeth
increaed IgE
Derm findings (ezcema)

Question 20

Pt with chronic Candida infections. Low IL-2 and INF-y. Defect?

Answer 20

Chronic mucocutaneous candidiases

Question 21

Young pt with chronic diarrhea. Bacterial, fungal and viral infections. Absent thymic shadow.

Answer 21

SCID (no T or B cells)

Question 22

Pt with increased AFP and decreased IgA, IgG and IgE. Defect?

Answer 22

Ataxia-telangiectasia. ATM gene mutation, bad repair.

Question 23

Pt with bad balance, spider angiomas, and multiple respiratory infections. Defect?

Answer 23

Ataxia-telangiectasia. ATM gene mutation, bad repair.

Question 24

Pt multiple pyogenic infections. Decreased neutrophils, decreased IgA, IgG and IgE. Defect?

Answer 24

Hyper IgM. CD40L defect (can’t class switch)

Question 25

Pt with low platelets, rash and multiple infections.Labs will show? Defect?

Answer 25

Wiskott-Aldrich. WASP gene (T-cell cytoskeleton defect)

Question 26

Pt with Increased IgE, IgA, decreased IgM. Defect?

Answer 26

Wiskott-Aldrich.

Question 27

Defect in LFA-1 integrin, CD18

Answer 27

Leukocyte adhesion deficiency

Question 28

Pt with recurrent bacterial infections. No pus.

Answer 28

Leukocyte adhesion deficiency

Question 29

Pt who's umbilical cord took a long time to come off?

Answer 29

Leukocyte adhesion deficiency

Question 30

Giant granules in neutrophils.

Answer 30

Chediack-Higashi

Question 31

Organ transplant. Organ becomes cyanotic and mottled almost instantly? Leads to?

Answer 31

Hyperacute rejection. HSR II. Leads to ischemia and necrosis

Question 32

Organ transplant. Pt becomes sick a few weeks later? Histo?

Answer 32

Acute rejection. Vasculitis with interstitial lymphocytic infiltrate.

Question 33

Organ transplant. Pt becomes sick a few months later? Histo?

Answer 33

Chronic. Obliterative vascular fibrosis. HYPOCELLULAR. irreversible.

Question 34

Organ transplant. Pt comes in with maculopapular rash, jaundice, hepatosplenomegaly, and diarrhea.

Answer 34

Graft vs Host.

Question 35

Pt with chronic renal failure presents with carpal tunnel and joint pain. Dz?

Answer 35

B2-microgobulin amyloid. Seen in dialysis pts.

Question 36

Heriditary amyloid that affects heart and brain?

Answer 36

ATTR

Question 37

Cause of amyloid if found in: 1) heart 2) thyroid 3) pancreas 4) brain 5) pituitary

Answer 37

``` 1-ANP 2-Calcitonin 3-Islet amyloid protein 4-B-amyloid (APP) 5-Prolactin ```

Question 38

Most common benign tumor in male/female?

Answer 38

lipoma/leiomyoma

Question 39

Movable mass at angle of jaw?

Answer 39

mixed tumor

Question 40

Female with RLQ pain with radiographic calcifications?

Answer 40

tertatoma with teeth

Question 41

Pt with Tuberous sclerosis - increased risk of what cancer?

Answer 41

Giant cell astrocytoma renal angiomyolipoma Cardiac rhabdomyoma

Question 42

Bombesin - marker for?

Answer 42

Neutroblastoma Lung cancer Gastric cancer

Question 43

hyperacute vs acute rejections - main cells?

Answer 43

Abs (B-cells) vs CTL (T-cells)

Question 44

Pt has disease with a failure for Th1 to produce INF-y vs failure of Th1 to respond.

Answer 44

Hyper-IgE vs IL-12 deficiency