IMMUNOLOGY

Question 1

Cyclosporine : MOA/ INDICATION/TOXICITY

Answer 1

Inhibit IL-2 & blocks T cell activation
Psoraisis, RA
Nephrotoxic(vasocontriction), gingival hyperplasia and hirsutism, HTN, hyperK and gout

Question 2

Tacrolimus: MOA/ Indication/ Toxicity

Answer 2

Calcineurin Inhibitor, binds FK506 binding protein, blocks IL-2 by blocking T-cell
Immunosuppresiona after organ transplant
Increase risk of diabetes, nephrotoxicity

Question 3

SIRolimus/ Rapamycin: MOA/ Indication/ Toxicity

Answer 3

mTOR inhibitor, binds FKBP
Kidney transplant prophylaxis
panSIRtopenia

Question 4

Basiliximab: MOA/Indication/ Toxicity

Answer 4

Monoclonal Antibody blocks IL-2R
Kidney transplant
Edema, HTN, Tremor

Question 5

Antithymocyte globulin derived from? MOA? Adverse effects (AE)

Answer 5

Rabbit serum
targets T cells
Potent immunosuppresion for renal transplant
Serum sickness, leukopenia and thrombocytopenia

Question 6

Serum sickness

Answer 6

Type 3 hypersensitivity –> 3 things stuck togethar–> Antigen-antibody-complement
in Serum sickness, anti bodies to foreign proteins produced after 1-2 wks.
Sx: fever , urticaria, arthralgia, proteinuria
serum C3, C4 low due to complement activation and deposit

Question 7

X-Linked agammaglobunemia : Defect/Presentation/ findings/ Contraindications?

Answer 7

BTK gene, X linked , no B cell maturation
Sinopulmonary and enteric infections after 6 months
No B cells or Igs in blood, absent LNs and tonsils, low CD19
live vaccines contraindicated

Question 8

Selective IgA def: Presentation/ Findings

Answer 8

Airway , GI (giardia) infections
can cause false negative celiac disease test and false positive prego test

Question 9

CVID

Answer 9

Defect in B-cell differentation
Puberty
Increased risk of autoimmune diseases, lymphoma, sinopulmonary infxns
low plasma cells, Igs

Question 10

Leukocyte adhesion deficiency: Defect/ Clinical/ Labs

Answer 10

defect in CD18 containing integrins
impaired leukocyte adhesion and endothelial migration
skin and mucosal infxns (cellulitis, peridontitis) without pus
delayed umbilical cord separation and wound healing

Question 11

complement deficiency increases risk of 3?

Answer 11

Neisseria, haemophilius, streptococcus (all encapsulated)

Question 12

3 types of defects in SCID

Answer 12

1. Adenosine deaminase(AR)
2. Il-2R gamma chain (XR)
3. RAG mutation –> VDJ recomination defect

Question 13

Chediak Higashi : Defect/ findings

Answer 13

Defect in lysosomal trafficking regulator gene (AR)
microtubule dysfunction in phagosome- lysosome
Albanism, neuropathy, pancytopenia, giant granules

Question 14

CGD Defect/ Dx/ Tx?

Answer 14

Defect of NADPH oxidase–> low ROS–> Increased catalase positive infxns, granulomas
Dihydrohydoamine test mein low green flourescense
nitroblue dye fails to turn blue
Rx: PPX- TMP-SMX, Itraconazole, IFN-Gamma
Hematopeiic cell transplant is cure

Question 15

BT reactions names?

Answer 15

Allergic
Acute hemolytic
Febrile Non hemolytic
Transfusion related acute lung injury
Delayed hemolytic

Question 16

BT allergic reaction/ Pathogenesis/ Timing/ Clinical presentation

Answer 16

against plasma protein in transfused blood
IgA deficient people should be careful
within 2-3 hrs

Question 17

BT acute hemolytic vs Febrile nonhemolytic reaction vs delayed hemolytic

Answer 17

Acute hemolytic- Type 2, due to ABO incompatibility, reaction within 24 hrs
Febrile Nonhemolytic - cytokines created by donor WBCs, within 1-6 hrs
Delayed hemolytic- extravascular hemolysis due to old encountered foreign antigens, within 1-2 wks

Question 18

Transfusion related acute lung injury

Answer 18

Neutrophils activated by antileukocyte antibody in the transfused blood–> increased permeability–> non cardiogenic pulmonary edema

Question 19

Post transplant bacterial infxn

Answer 19

> 1 month: Due to post-op complications or hospitalisation
1-6 months: Opportunistic pathogens due to high dose immunosuppression ( Cytomegalovirus, Aspergillus, EBV MTB)
<6 months: higher risk of Community acquired pathogens

Question 20

Types of Transplant rejection?

Answer 20

Hyperacute
Acute
Chronic
GVsH

Question 21

Hyperacute & acute Transplant rejection : Timing/ Pathogenesis/ Features

Answer 21

Hyperacute: within minutes due to preformed antibodies (type2 activates complement)
- thrombosis in graft vessels–> ischemia and necrosis
Acute: Weeks to months or <90 days , due to antibodies forming after transplant or due to cellular response secondary to T- cell activation by donor MHCs
- vasculitis of graft vessels, reverse with immunosuppression

Question 22

Chronic transplant rejection

Answer 22

months to years,
dominated by arteriosclerosis
due to both Cellular and humoral components via APCs presenting donor peptides

Question 23

Graft vs Host disease

Answer 23

T cells from graft proliferate in host and attack the organ
due to HLA mismatch
type 4 reaction
Maculopapular rash, jaundoce, diarrhea, hepatosplenomegaly
Usually seen in BM or liver transplants
Irradiation of blood products important.

Question 24

CGD Infections?

Answer 24

Nocardia
Burkholderia (psuedomonas)
Staph
Serratia
fungus
All cause cavitary lesions

Question 25

Herditary Angioedema/ C1 inhibitor deficiency

Answer 25

Causes Increased Bradykinin–> edema of face, limbs, genitals, larynx, bowel wall
Due to stress or trauma
Self resolving
low C4 levels
Rx: C1 inhibitor concentrates, Bradykinin antagonist (Icatibant), Kallikrein inhibitor (Ecallantide)

Question 26

Bradykinin metabolised by?

Answer 26

ACE

Question 27

DTaP vaccinations contraindicated in?

Answer 27

Anaphylaxis after vaccination
encephalopathy, uncontrolled epilepsy, infantile spasms within a week of DtaP vaccine

Question 28

Anaphylaxis management (also in patients on BB?)

Answer 28

Epinephrine upto 3 doses–> IM preferred–> IV for severe cases
IV crystalloids, Trendelenburg position for Hypotension
Albuterol for bronchospasm
Glucagon for pts on BB