Practice Questions Flashcards

1
Q

_________enzyme is present ONLY in phagocytic cells of the immune system (mostly neutrophils) and converts _________ to _______.

A
  1. _________enzyme is present ONLY in phagocytic cells of the immune system (mostly neutrophils) and converts _________ to _______.
    a. Myeloperoxidase (MPO) MPO contains 2 Fe heme-like centers=green=pus
    b. H202 —> HOCl (Hypochlorous Acid: powerful toxin that destroys bacteria through halogenation and oxidation reactions)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

_________ allow cells to upregulate production of free radicals in response to infection (which causes damage to lipids, proteins, carbs, and DNA&raquo_space;> collateral damage: cell death/CA/disease.)

A

_________ allow cells to upregulate production of free radicals in response to infection (which causes damage to lipids, proteins, carbs, and DNA&raquo_space;> collateral damage: cell death/CA/disease.)

Respiratory Bursts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Three year old kiddo is brought into your office today with his third bout of pneumonia since January (you culture his sputum and later find the culprit to be aspergillus). Upon further examination, you also find oral thrush, which you treated him for three weeks ago when it developed after antibiotic treatment for his pneumonia. You suspect a rare X-linked disease: Chronic Granulomatous Disease, may be underlying these weird recurrent infections. As a result you are more worried about him getting infected by staph aureus or nocardia than you are about a streptococcus infection. Why?

A

a. CGD is a deficiency of NADPH oxidase in phagocytes. Aspergillus, staph aureus and nocardia are catalase negative organisms = they degrade enough H202 into ROS that the human’s phagocytes can use those ROS against the bacteria in absence of the phagocytes’ own NADPH oxidase products.
b. Catalase Positive bacteria (eg streptococcus) degrade a minimal amount of H202, so human phagocytes without NADPH oxidase don’t have enough ammunition to destroy the bacteria.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

A lumberjack was injured near Cloquet [nice :)] when an enormous tree fell in an unsuspected fashion, pinning his right leg to the ground. The circulation in his leg is compromised. Since his leg isn’t broken, and he isn’t bleeding (besides a few scrapes), the lumberjack thinks that once his leg is unpinned, he will be “out of the woods.” But what is he at risk for?

A

a. Reperfusion Injury.
blocked blood flow —>decreased oxygen to tissues —> decreased ATP available —-> increased Na+ and Ca++
The reduced state of the electron carriers in the absence of oxygen PLUS the loss of mitochondrial ion gradients/membrane integrity leads to HUGE increase in Superoxide production when Oxygen becomes available again during reperfusion.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q
  1. So, you’re a Paneth Cell. Awesome. Tell me i) where are you located?; ii) what feature of the innate immune system do you wear like a badge of honor?; iii) what does this feature allow you to do?
A

a. i) In the small intestine; ii) you totally rock an ALPHA-Defensin known as Human Defensin (HD); This DEFENSIN enables you to increase cellular permeability and modulate the inflammatory response
i. Note: even though it’s categorized as an “alpha”-defensin, the other kids at school can’t make fun of you cause it’s still a BETA-pleated sheet, just like all the other Defensins, but yours is named “alpha” because the linking pattern of your cysteines is just a little different. You’re unique. And that makes you special.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q
2.	TLRs are activated by the following factor derived from fragments of the host:
 .	PAMP
a.	DAMP
b.	CHAMP
c.	LAMP
A

. Answer: DAMP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q
3.	Which TLR is the only one to NOT utilize the MyD88 cascade pathway?
 .	TLR5
a.	TLR9
b.	TLR3
c.	TLR1/6
A

. Answer: TLR3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q
  1. With respect to the various TLR cascade pathways, those that feature IRF3 yield expression of __a__, those with IRF7 yield expression of __b__, while the “mother of all immune system transcription factors” known as __c__ yields expression of __d__.
A

a IFN-Beta

b. IFN-Alpha
c. NF-kB
d. pro-inflammatory cytokines

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q
  1. __a__ are the most important players in the acute inflammatory response, while __b__ govern chronic inflammation. Regarding the allergic response, __c__ are involved in the immediate phase of an allergic reaction, whereas __d,e__ are involved in the late phase.
A

a. PMNs (Neutrophils)
b. Macrophages
c. Mast Cells
d. Eosinophils
e. Basophils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q
  1. During myelopoeisis, a Monocyte can be differentiated into a(n) _____ if exposed to _____.
A

. TWO possible answers:
. Macrophage; M-CSF + GM-CSF
i. Dendritic Cell; GM-CSF + IL-4

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q
  1. What are the THREE cells capable of antigen presentation (APCs)?
A

. Dendritic Cells, Macrophages, B Cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q
  1. You are on clinical rounds when you meet an 18 month old female brought in by her mother. The child was recently diagnosed with MHC Class II deficiency. What type of cells do you expect to see depleted in the child? This is the result of the inability of what type of selection?
A

a. The child would be deficient in CD4+ cells as a result of the lack of positive selection occuring in the thymus due secondary to MHC class II deficiency. If the thymus is deficient in MHC Class II, the maturation of CD4 T cells will not occur. CD8 T cells will be unaffected as they bind to MHC class 1 molecules.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q
  1. The child was not diagnosed with MHC Class II deficiency until 6 months of age when she presented with opportunistic pneumonia. Why was the child’s health protected, despite having the autosomal recessive MHC Class II deficiency, until 6 months of age?
A

. The child was protected via receiving IgG from her mother through passive immunity. Passive immunity typically only lasts 6 months before the mothers IgG starts to deplete from the child’s system, and thus 6 months is the age where many hereditary immunological deficiencies first present.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q
  1. Why is the type of immunity described in number 2 “faster”?
A

. IgG is directly passed to the child through passive immunity. Via active immunity, IgM is the first antibody to appear as it is better at complement activation in response to new antigenic exposure. IgG is generally not made for a week or two in active immunity.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q
  1. The mother is also worried about her daughter having symptoms of asthma. What is causing her daughter’s bronchoconstriction? What hypersensitivity type is this?
A

. This is a type 1 hypersensitivity reaction known for its fast onset in response to environmental trigger. It’s caused by IgE attaching to mast cells, and when this pairing binds to antigen, degranulation occurs, resulting in the release of histamine from mast cells in addition to leukotrienes and prostaglandins-> bronchoconstriction.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q
  1. You are a scientist at the NIH studying a rare disease caused by a mutation in the J-chain. (This is in the antibody structure, we are not talking about the J region of DNA that goes through VDJ recombination). What Ig isotypes will this mutation affect? And subsequently what parts of immune function will lose effectiveness?
A

a. Isotypes IgM and IgA will be affected because they use the J-chain in their structure to hold things together
i. IgM is the first Immunoglobulin to appear in serum at the onset of infection or after a vaccine. Good sign of current infection
ii. IgA has a strong protective role in secretions on mucous membranes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q
  1. What is the valence of IgM? What would be the valence of IgM if you were able to remove all the the light chains and not disrupt anything else?
A

a. Valence = number of antigenic determinents (AKA epitopes) that an antibody can theoretically bind
i. IgM is composed of 5 basic units with 2 binding regions each. This gives it a valence of 10
ii. Remember that in order to bind to an epitope the antibody must have a V region from BOTH the light and heavy chains to make the combining site. Without the light chains as part of the combining sites, this molecule will bind to no antigens and have a valence of 0.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q
  1. There are 10 isotypes of immunoglobulins: 4 IgG, 2 IgA, 2 IgM, 1 IgD, and 1 IgE. These are based on slight difference in amino acid sequences of their heavy (H) chain constant (C) regions. Immunoglobulins can be further differentiated into allotypes and idiotypes. What determines allotypes and idiotypes?
A

. Allotypes - Inherited minor allelic differences in the Ig sequences
. possible genetic determinant in immune function
a. Idiotype - Differences in individual combining sites (the combined light and heavy chain variable regions) also known as the complementarity-determining region (CDR).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q
  1. Which immunoglobulin is important for resistance against worms and parasites and would underly an individual’s allergies?
A

. The would be IgE… Aller-gEEE!

20
Q
  1. Put these antibodies in order in regards to relative concentrations in normal blood serum: IgG, IgM, IgE, IgD, IgA
A

. MOST ← IgG, IgA, IgM, IgD, IgE → LEAST

21
Q
  1. What combination of these facts is correct about EBV
    A. HHV6, Lytic in skin, No latency, treatment: Supportive
    B. HHV5, Lytic in nasopharynx, Latent in T-Lymphocytes, treatment: Gancyclovir
    C. HHV8, Lytic in oropharynx, Latent in epithelial cells, treatment: chemo
    D. HHV4, Lytic in oropharynx, Latent in B-Lymphocytes, treatment: Supportive
A

Answer D

22
Q
2.	What malignancy is not associated with EBV:
A. Hodgkin Lymphoma
B. Burkitt Lymphoma 
C. Nasopharyngeal Lymphoma
D. Chronic Myeloid Leukemia
E. Non-Hodgkin Lymphoma
A

Answer: D

23
Q
  1. 3 year old boy presents to urgent care appearing very ill with fever and sore throat. Upon examination you can palpate the spleen and liver. His lymph nodes are enlarged, and you see a tinge of yellow discoloration in his sclera. His monospot test result was heterophile Antibody +, and blood smear reveals reactive lymphocytes including Downey cells (diagnostic for EBV). A. Why is the presentation so severe in this child? B. What his his prognosis? C. And why need his mom not worry about his sisters contracting this severe illness?
A

A. He shows symptoms of Duncan Disease, also known as X-Linked Lymphoproliferative Disease. Due to an inherited mutation in SAP (SLAM associated protein) adapter, the child has an exaggerated response to EBV resulting in fulminating infections. Typical age of presentation is 3 yo.
B. IF he survives this episode, he will continue to have lymphoproliferative disorders (s.a. lymphomas) and dysgammaglobulinemias. Most do not live for more than 1-2 months after diagnosis.
C. This is X-linked recessive and almost exclusively affects males.

24
Q
  1. How does EBV gain access to cells in establishing latency?
A

Binds CD21 on B-cells – enters via endocytosis. Latent form has circular DNA. (when lytic, DNA is linear.)
note: VCA-IgG indicates PAST infection. VCA-IgM indicates Acute infection.

25
Q
  1. List the oncogenes associated with EBV and the effect of each:
A

LMP1 = CD40 analog—-> transcription of NFkB genes
LMP2 = BCR analog —-> transcription of fos or jun
EBNA3 —-> activates cyclin D1 which activates RB (retinoblastoma proten)

26
Q
  1. You draw blood from a pregnant patient and measure antibody isotypes in the serum. What relative antibody concentration do you expect to find and what are their half-lives?
    a. Which antibody type has probably crossed the placenta and reached her fetus?
    b. Name the antibody isotope best at
    i. complement fixation & bacterial lysis,
    ii. mast cell/basophil degranulation.
    iii. antiviral activity
    iv. toxin neutralization.
A

. IgG should have the highest serum concentration; IgD should be very low. IgG has the highest serum half-life [8-23 days], followed by IgA [6], IgM [5], IgD [3], and IgE [2.5].

a) IgG only one that can Get across the placenta into fetus.
b) Best at:
i. coMpleMent fixation & bacterial lysis – IgM [IgG & IgA both try to help w/bacterial lysis]
ii. mast cell/basophil dEgranulation – IgE
iii. Antiviral Activity – IgA (IgG & IgM both try this…)
iv. toxGin neutrAlizAtion – tie between IgA & IgG :P

27
Q
  1. Arrange the domains of the heavy chain family according to # of variations.
    a. Which of the 3 (V/D/J) is not found in the light chain?
A

2) V>J>D

a. V & J are found in the light chain, D is not.

28
Q
  1. You find a mouse escaped from a high-security experimental facility, and discover that its RAG-1 and RAG-2 have been knocked out. What are RAG-1 & RAG-2, what do they do, and what does this mean for the mouse’s future?
A

3) RAG-1 & RAG-2 recombinases are the enzymes in charge of antibody & Tcell receptor DNA recombination. They bind splice signals to the right of a D segment and the left of a J segment, pull them together, and then cut and splice. Then they look for a splice sequence to the right of a V segment & repeat. With RAGs knocked out, this poor mouse can’t make B or T cells. (If this happened to a human it would be called Omenn Syndrome – rare.)
Also RAGs recognize, cut, pull together…but don’t always join perfectly, due to action of exonucleases & TdT (adding random nucleotides) – create Imprecise junction, creating completely random diversity! This is SOMATIC variation.

29
Q
  1. When a faulty rearrangement is detected during recombination, what special process can occur if the RAG recombinases are still active? What happens once they are no longer active?
A

4) Receptor editing. If not quick enough & the RAG recombinases are no long active, when nonsense codons are produced the cell can try again using the other allele. If neither works, the cell dies.
Once the heavy gene has been made, the cell then uses one of the kappa alleles to try to make a light chain match. If the first does not work, it then tries the other kappa allele, then the lambda allele, then the other lamda allele. If none match, no receptor is made & the cell dies.

30
Q
  1. What is the progression of Ig expression & B cell maturation? At what processing level do the changes occur?
A

5) Ig expression always start with IgM –> Hi IgM/lo IgD –> lo IgM/hi IgD –> mature non-activated B cells can express both. [switch at mRNA processing level]
Once activated, B cells switch from membrane-bound IgD & IgM to secretory IgM. [switch at mRNA transcript level – from polyA2 to polyA1]
Can then switch from IgM to IgG, and from IgM/IgG to IgE or to IgA. [switch at DNA rearrangement level – can’t go back to making IgM once switched to IgA]
Without T-cell help, only IgM responses are possible. In all cases, the L chain and the VH domain stays the same but the C region of the H chain changes.

31
Q
  1. How does somatic hypermutation occur?
A

) Activation-Induced (Cytidine) Deaminase (AID) converts random cytosines in the CDR gene regions to uracil, making uracil: guanine mismatches which, once repaired, mostly result in single-base substitution mutations. Mutates on purpose, gets random mutations that are better or worse.

32
Q
  1. Where is complement located?
    a. Lymph
    b. Urine
    c. Red Blood Cells
    d. Plasma
A

i. (Answer: D, but also interstitially and in secretions)

33
Q
2.	Which component of plasma is found in the highest concentrations?
 .	C1qr2s2
a.	C3
b.	C9
c.	C5
A

. (Answer: a, 1200 ug/mL! wowza!)

34
Q
3.	Which C3 Convertase is utilized in the alternative pathway?
 .	C4b2aC3b
a.	C3bBb
b.	C4b2a
c.	Convertase III
d.	Properdin
e.	Factor H
A

. (Answer: B! Although option C is also a C3 convertase, it acts in the classical pathway activation. Option B is in the alternative pathway, and is stabilized by option E, properdin, aka component P)

35
Q
4.	What occurs in ALL learned pathways?
 .	Antibody recognition
a.	Amplification
b.	Factor H fluid phase inhibition
c.	C4a binds to activator surface
A

. (Answer: a! Remember, antibody recognition is only a characteristic of the classical pathway (IgG [2] and IgM), alternative and MBL pathways do not require specific antibodies. Amplification occurs in all pathways. Factor H is an inhibitor in the alternative pathway, binding to the C3bBb C3 convertase. C4a floats away while C4b is the binding component.)

36
Q
5.	\_\_\_\_\_\_\_\_\_ is a major binder to CR1.
 .	CR0
a.	C5
b.	C2a
c.	C3b
A

. (Answer: c! CR1 readily binds C3b to block C3 convertase formation and promoting phagocytosis of whatever C3b is bound to [i.e. bacteria].)

37
Q
6.	So you see a new patient of yours, a young man in his early 20’s. He’s in for evidence of a 4th sinopulmonary infection since he moved to your small rural town 6 months ago. You think, holy crap, this seems like an immune complex disease. Deficiency in what complement component is your patient most likely experiencing? *Yay for two step questions!*
 .	C1qr2s2
a.	C3
b.	C4
c.	C3bBb
d.	C2a
A

. (Answer: b! Highest incidence of IC is caused by C4 deficiency. NOTE: meningococcal infections are most related to deficiency in the terminal lytic pathway components, which would be C5-C9)

38
Q
  1. You’re driving along Superior Street and witness a near-fatal car accident in front of you. After approaching the victim, you notice his blood type is tattooed on his arm, AB-. You know he has lost enough blood that he will not make it to the hospital, and you happen to have a tube and two sterile needles in the back seat of your car. You perform a field transfusion because you are blood-type:
    a. AB+
    b. O+
    c. B-
    d. A+
A

i. (Answer: C! Remember, type AB is a universal recipient, but if they are Rh negative, receiving Rh+ blood would be detrimental.)

39
Q
2.	Later, you’re doing rounds at the hospital and find out that the blood type tattooed on his arm was actually in memory of his mother, who had blood type AB-. He, however is adopted and turns out to be a different blood type. A forward-type test is conducted to determine his true blood type. Vials of his blood were mixed with  anti-A, anti-B and anti-Rh antibodies, and each A/B vial remained homogenous or nonclumped, however the Rh vial did clump up. This reveals his true blood type is \_\_\_.
 .	O+
a.	AB+
b.	B-
c.	Bombaii type
A

. (Answer: .! Remember that clumping of blood cells during a forward-type test reflects that the anti-whatever antibodies bind the “whatever” receptors and clump up when the AHG is added, so it is the “whatever” blood type if it clumps because of the presences of the “whatever” antigens.) ← that might not make sense, but you should probably know what i mean.

40
Q
  1. Uh oh. Luckily, due to the good samaritan law, you are not accountable because you were trying your best to save his life and were able to get him to the hospital. However, his reaction due to receiving an unmatched blood transfusion is most likely:
    . Allergic Transfusion Reaction
    a. Circulatory Overload
    b. Delayed Hemolytic Transfusion Reaction
    c. Acute Hemolytic Transfusion Reaction
A

. (Answer: c! Although all may occur in this patient because he could be allergic to donor plasma [option A], could have received too much blood in the field [option B], or could have a reaction to non-ABO antibodies [option C], option D is most likely because of the incorrect ABO match between donor and recipient)

41
Q
  1. This guy’s luck just sucks. He received an appropriately typed blood transfusion, however, he experienced another transfusion reaction. This time, reacting against the donor’s white blood cells, with symptoms of fever, headache, nausea, and chest pain. What is the most appropriate treatment for this reaction? two-step, woo!
    . Tylenol, and administration of leukocyte-reduced components
    a. STOP transfusion immediately
    b. Antihistamines
    c. Steroids
    d. Aggressive antibiotic therapy
A

. (Answer: .! This is a febrile transfusion reaction. Although I suppose stopping the transfusion right away would probably be acceptable, the tylenol and leukocyte-reduction is what Krafts listed as treatment on her ppt. Antihistamines would be for an allergic transfusion reaction, steroids I made up, and antibiotics would be for a bacterial infection that was in the donor blood.)

42
Q
5.	A woman comes into your office to confirm her pregnancy after taking a TrueBlue home test. You do confirm that she is about 8 weeks pregnant. After finding out that her blood type is A-, and her husband, a geneticist is aware that he is homozygous Rh positive, you let her know that at 28 weeks she needs \_\_\_\_\_.
 .	3D ultrasound
a.	Fetal hemoglobin
b.	Rhogam administration
c.	Anti-B Ag
d.	Blood transfusion
A

Answer: b! Since her baby’s blood type is not Rh compatible with hers, she will need Rhogam at 28 weeks and upon delivery.)

43
Q
  1. 32 weeks later, you deliver her baby, and need to give her the second Rhogam dose. How much do you give her?
    . It depends how much she bled during delivery.
    a. It depends on the Schilling test results.
    b. It depends on if they baby is Bombaii blood type.
    c. It depends on how much the baby and mom’s blood mixed.
A

. (Answer: c! You use the Kleihauer-Betke test, taking a blood smear of the mother’s blood, expose the smear to acid bath, removing HbA, stain the smear, and baby cells are pinker. If there are more baby cells present, you administer more Rhogam, if there are less, you give less.)

44
Q
  1. What changes in your interpretation of labs if the RDW value is found to be REALLY high?
A

a. This indicates that there is a large MIX of both large and small cells, so you may still have a microcytic/macrocytic anemia even though the AVERAGE (MCV) is reported normal. In the example given in class, the patient was suffering from IDA (a microcytic anemia) which was masked by his use of a macrocytic drug known by 6-mercaptopurine.

45
Q
2.	A middle-aged patient walks in with an EXTREMELY microcytic MCV value, but only moderate anemia.  These just don’t add up… EXCEPT, because you’re a thoughtful hematologist, what do you suspect and what test helps you confirm your suspicion?
 .	IDA, Bone Marrow 
a.	Warm AIHA, IgG+ DAT
b.	B-Thalassemia, Hb Electrophoresis
c.	Cold AIHA, IgM+/C3+ DAT
A

i. Answer: b

ii. Note: B-Thalassemia minor is usually not diagnosed until adulthood

46
Q
  1. You’ve got a mysterious patient who can’t remember her own name, and whose labs indicate a macrocytic (elevated MCV) hemolytic (elevated bilirubin, low haptoglobin, jaundiced) anemia (low Hb), complexed with possible autoimmune thyroiditis (elevated TSH). After calculating her true “reticking” value using the RPI, you realize that she is not reticking adaquately. You also notice Vitiligo on physical exam. What do you suspect, and what treatment do you administer?
    . Iron Overload, Fe Chelation
    a. Warm AIHA, prednisolone taper with possible splenectomy if refractory
    b. Stage 4 bladder cancer, love and support
    c. Pernicious Anemia, B12 infusion followed by monthly IM injections
A

. Answer: c

47
Q
4.	Diagnosis of WARM AIHA requires DAT+ \_\_\_\_, while diagnosis of COLD AIHA requires DAT+\_\_\_\_?
 .	anti-IgG, anti-IgM/C3
a.	anti-IgM/C3, anti-IgG, 
b.	antidisestablishmentarianism
c.	anti-IgG1, anti-IgG2
A

. Answer: .