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MOD > Immunodeficiencies > Flashcards

Immunodeficiencies Flashcards

1
Q

Where does B cell maturation take place

A

Bone marrow

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2
Q

Where does T cell maturation take place

A

Thymus

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3
Q

What is the primary site of hematopoiesis starting in embryonic period

A

BOne marrow

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4
Q

What type of T cells do you find in the cortex

A

immature t cells coming from bone marrow

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5
Q

Where are hassall corpuscles located?

A

in medulla of thymus

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6
Q

Where do APCs interact with T /B cells

A

lymph nodes

T cells in paracortex
B cells in germinal center

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7
Q

Viruses and tumors bind to MHC class ________

A

Class 1 : CD8+

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8
Q

Bacteria, allergens , exogenous pathoogens bind to MHC class ________

A

Class 2 : CD4+

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9
Q

What kind of cells reside in peripheral organ /tissues?

A

Mature B/T cells

lymph nodes
spleen
mucosa associated lymphoid tissues

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10
Q

MHC class 1 is present on ____________

MHC class 2 is present on _____________

A

all nucleated cells

APCS

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11
Q

What is the importance of HLA polymorphisms?

A
  1. important when considering organ transplants

2. associated with certain autoimmune diseases

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12
Q

Cell mediated immunity requires ________ cell proliferation

A

T cell prolifeation, differentiation, migration , killing

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13
Q

If T helper cells are no longer intact what part of B cell differentiation does this efffect?

A

Isotype switching
affinity maturation

In general something wrong with T helpers WILL EFFECT HUMORAL IIMMUNITY (AB mediated)

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14
Q

which Ig antibody protects the mother but does not cross the placenta and cannot protect the child?

A

IgM

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15
Q

Lack of which Ig increases susceptibility to mucosal infections?

A

IgA

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16
Q

Which Ig , if measured, provides information about presence of ongoing infection

A

IgG

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17
Q

Virus infected cells have what property that contribute to destruction of cells?

A

decreased expression of MHC class 1

MHC class 1 inhibits NK cells

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18
Q

What DIRECTLY contribute to a patients presentation of angioedema?

A

degranulation of mast cells. ( this causes what you are able to see)

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19
Q

WHat constitutes a late reaction TYpe 1 hypersensitivity?

A

inflammatory cells: eusinophils, basophils
epithelial damage
bronchospasm

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20
Q

What constitues an immediate type 1 hypersensitivity

A
  1. bronchoconstriction,
  2. edema (vascular leakage)
  3. muscle spasm (increase bowel peristalsis)

from mast cell degranulation

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21
Q

A child comes in after eating a cherry 3 hours ago and mom says he has trouble swallowing. What histologic finding do you expect to find?

A

Eusinophilic

- this is eusinophilic esophagitis
Type 1 (Late) Hypersenstivity
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22
Q

What are the 3 mechanisms of type 2 hypersensitivity?

A
  1. inflammation
  2. opsonization /phagocytosis
  3. cellular dysfunction (inhibiting receptors/ acitivating)
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23
Q

Which type hypersensitivity results in low cell count?

A

Type. 2

ex. autoimmune hemolytic anemia, autoimmune thrombocytopeia

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24
Q

What is the mechanism of Autoimmune hemolytic anemia and autoimmune thrombocytopeia?

A

Opsonization/ phagocytosis (LOW CELLS)

TYPE 2 Hypersensitivty

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25
Q

What are the 3 type 2 hypersensitivity diseases caused by inflammatory mechanism?

A
  1. vasculitis
  2. good pasture
  3. acute rheumatic fever
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26
Q

Describe type 2 hypersensitivity inflammatory mechanism ?

A
  1. Fc receptor / complement bind

2. then, neutrophil enzymes , and ROS release causing tissue injury

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27
Q

Which of the following is a result of protein targeting in the basement membrane of kidney glomeruli and lung alveoli?

  1. Good pasture
  2. rheumatic fever
  3. systemic lupus
  4. asthma
  5. acute sclerosis
A

Good Pasture. (Type 2 )

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28
Q

Rheumatic fever is a cumulative inflammation and scarring of the heart valves resulting from an abnormal immune response due to molecular mimicry between ________ and ____________

A 
streptococcal protein and myocardial proteins 
Type 2 (inflammatory)
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29
Q

Which hypersensitivity results in disrupted endocrine signaling and neural signaling?

A

Type 2 (mechanism of cellular dysfunction)

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30
Q

Which diseases are caused by cellular dysfunction type 2 hypersensitivity?

A

Myasthenia gravis ( inhibits ACH binding)
GRaves (stimulation of TSH)
insulin resistant diabetes ( inhibits insulin binding)

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31
Q

Ag-Ab complexes produce tissue damage mainly by causing inflammation where?

A

At site of DEPOSITION (type 3)

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32
Q

immune complex disorders tend to be ____________( Systemic / local) and favor what 4 locations of the body?

A

SYSTEMIC

joints (arthritis)
skin
kidneys (glomerulonephritis)
small blood vessles (vasculitis)

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33
Q

is systemic lupus an ACUTE or CHRONIC serum sickness? WHy

A

CHRONIC
it is caused by persistenc ab response to autoanitgents

acute would be a result of a single LARGE exposure to antigen

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34
Q

A smooth and linear type immunofluorescence would confirm which of the following?

A. SLE
B. asthma 
C. type 1 diabetes
D. automimmune hemolytic anemia
E.  food allergy
A

SMOOTH /LINEAR = TYPE 2 hypersensitivity

This is D.

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35
Q

Which of the following would show grainy, patchy immunofluorescence?

A. SLE
B. vasculitis
C. good pasture
D. Asthma

A

GRAINY= TYPE 3

This is A.

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36
Q

A post streptococcal cross reactive antibodies in theKIDNEY would cause what type of immunoflurorescence?

A

GRAINY. (TYPE 3)

Antibodies cause DEPOSITION in the kidney

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37
Q

post streptococcal cross reactive antibodies in the HEART would cause what type of immunoflurorescence?

A

SMOOTH / LINEAR (type 2)

the antibodies act DIRECTLY ON MYOCARDIUM

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38
Q

WHich _________(CD4 / CD8) causes DIRECT tissue injury?

A

CD8

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39
Q

Granulomatous inflammation is a hallmark of _________

A

Type 4 hypersensitivity

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40
Q

Which _________(CD4 / CD8) causes mediates formation of immune granulomas?

A

CD4 that release TNF / INF —-> recruit macrophages

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41
Q

Chronic arthritis and destruction of articular cartilages is caused by what mechanism ?

A

T cell mediated inflammation

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42
Q

What is the cause of local swelling and induration following the administration of mantouxtest?

A

sensitized t cells start inflammatory response (TYpe 4)

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43
Q

Type 1 diabetes and rheumatoid arthritis are both type ______

A

4

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44
Q

What is a rare local effect of vaccination?

A

Arthus reaction (type 3 )

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45
Q

Th1 cells releasing cytokines and activating marcrophages produces what kind of immunofluorescence?

A

Type 4 hypersensitivty

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46
Q

What 3 requirements to truly define as autoimmune ?

A
  1. presence of immune rxn to specific SELF antigen or SELF tissue
  2. rxn is NOT sECONDARY to tissue damage but rather the primary cause
  3. abscence of another well defined disease
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47
Q

THe process by which T cells die of APOPTOSIS after selecting for self reactivity is called _______ tolerance?

A

CENTRAL

occurs in thymus

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48
Q

THe process by which B cells die of APOPTOSIS or undergo RECEPTOR EDITING after selecting for self reactivity is called _______ tolerance?

A

CENTRAL

in bOne marrow

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49
Q

What are the 2 main mechanisms of peripheral tolerance?

A
  1. anergy (failure to respond)

2. suppression by T regs

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50
Q

Tumors and viruses can use ______ and _____ receptors to avoid immune destruction?

A

CTLA

PD1

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51
Q

Patients with Gene knockout of CTLA and PD1 develop__________

A

autoimmune diseaseG

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52
Q

Gene knockout of IL-2 would lead to __________

A

autoimmune disease

b/c it stimulates T-regs and without them you cant prevent immune rxns against self antigens

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53
Q

Gene knockout of FOX p3 would lead to ___________

A

autoimmune disease OR fetal rejection

b/c it is needed for maturation and maintence of TREGS

AND

plays a role in suppressing mothers adverse reaction to paternal antigens of the fetus

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54
Q

What are the functions of TREGS

A

cytokine immunosuppression ( IL-10 , TGF B)

CTLA 4 inhibition

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55
Q

What is AIRE critical for?

A

(thymus) deletion of immature Tcells that attack SELF

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56
Q

Immune dysregulation, polyendocrynopathy , enteropathy , Xlinked is caused by _____________

A

IPEX : deletion of FOxP3

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57
Q

What disease is associated with Class 1 HLA B27

  1. high tsh levels
  2. hereditary inflammatory joints in the spine
  3. keratosis of the oral mucosa
  4. malar rash
A
  1. Ankylosing spondylylitis
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58
Q

INability of paneth cells in intestinal epitheilium to kill microbes is associated with polymophisms in ________

A

NOS 2 gene (CHRONS disease)

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59
Q

Rheumatoid arthritis and type 1 diabetes is associated with polymophisms in ________

A

PTNPN22 gene

results in excessive lymphocyte production

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60
Q

What two tests are diagnostic for SLE

A

anti DS DNA

Anti smith

61
Q

Homogenous staining of ANA is consistent with what autotimmune disease

A

SLE

62
Q

speckled staining of ANA is consistent with what autotimmune disease

A

Sjorgens

63
Q

Nucleolar staining of ANA is consistent with what autotimmune disease

A

systemic sclerosis

64
Q

What genetic predispostiion increases risk for SLE?

A

HLA -DQ

65
Q

List 4 other symptoms other than malar or discoid rash for SLE?

A

arthritis
oral ulcers
renal disorder
photosensitivity

66
Q

What lab test is important to do routinely for SLE patient?

A

CBC due to how it affects hematology and renal

67
Q

What environmental factor is involved in pathogensis of SLE?

A

UV light

68
Q

WHat kind of staining would be seen with diffuse lupus nephritis and why?

A

grainy due to immune complex formation

69
Q

What sxs present with diffuse lupus nephritis?

A

URINALYSIS

proteinuria
hematuria

70
Q

What type of cell can be found circulating in a patient with SLE?

A

L-E cell

a neutrophil or macrophage that ingests the nucleus of a damaged cell

71
Q

What are 2 cardio complications of SLE?

A

Libman Sacks endocarditis

coronary artery disease (possible contribution from antiphopholipid ab syndrome?

72
Q

positive ANA, discoid rash, (+) immunofluoresc. = ____

A

discoid lupus

73
Q

(+) arthralgias, (+) ANA, discoid rash, (+) hematologic disease, (+) immunofl. = _____________

A

drug induced lupus

74
Q

If you suspect drug induced lupus you would see a positive test for __________

A

anti- histone Ab

75
Q

48-year-old female presents to your office with a 1-year history of dry eyes and difficulty swallowing. She complains of blinking frequently and of eye strain while using her computer at work. She also reports stiffness in her knees and lower back. Past medical history is unremarkable and she does not take medications. She denies cigarette or alcohol use. Family history is notable for Hashimoto’s thyroiditis in her mother. Physical exam shows dry oral mucosa and enlargement of the parotid glands. Which of the following serologies is likely to be positive in this patient?

A. Anti-SS-A
B Anti-CCP
C. Anti-dsDNA
D. Anti-Smith
E. Anti-Jo-1
A

A.
anti Ro/SS A

anti La/SS

76
Q

What are 2 complications of Sjorgens?

A

lymphoma *because it is pathogenesis is mediated by T / B cells

pulmonary fibrosis

77
Q

What is eessential for dx of sjorgens

A

BIOPSY of lip ( to examine minor salivary glands)

78
Q

A primary autoimmune phenomenon that results in damage. As a result of this damage, the “roof” is lifted off of the underlying basement membrane, exposing tissues with their own antigens that are now the subject of a secondary attack. What is this concept

A

Epitope Spreading. (ex. lichen planus)

79
Q

A 35-year-old woman comes to your office with a variety of complaints. As part of her evaluation, she undergoes laboratory testing which reveals the presence of anti-centromere antibodies. All of the following symptoms and signs would be expected to be present EXCEPT:

  1. Pallor, cyanosis, and erythema of the hands
  2. Calcium deposits on digits
  3. Blanching vascular abnormalities
  4. Hypercoagulable state
  5. Heartburn and regurgitation
A

4.

CREST SYNDROME

Calcinosis
Raynauds
Esophageal dysfucntion
Telangiectasis
Sclerdactyly
80
Q

A 35-year-old woman comes to your office with a variety of complaints. As part of her evaluation, she undergoes laboratory testing which reveals the presence of anti-centromere antibodies. All of the following symptoms and signs would be expected to be present EXCEPT:

  1. Pallor, cyanosis, and erythema of the hands
  2. Calcium deposits on digits
  3. Blanching vascular abnormalities
  4. Hypercoagulable state
  5. Heartburn and regurgitation
A

4.

CREST SYNDROME

Calcinosis
Raynauds

Esophageal dysfucntion

Telangiectasis

Sclerdactyly

81
Q

Anti-centromere antibody= __________ (centromere staining)

Anti DNA topoisomerase = _________ (speckled staining)

A

CREST syndrome;

Systemic sclerosis

82
Q

_______ is affected first in Systemic sclerosis , _________ are affected LATER.

A

Skin. (sclerodactyly , raynauds)

Visceral ORgans: GI tract renal vascular disease, esophageal ulcers, pulmonary fibrosis

83
Q

What has better prognosis : (+) anti centromere or (+) anti DNA topoisomerase?

A

(+) anti centromere. (CREST syndrome BETTER PROGNOSIS )

84
Q

Mixed tissue disorder has high titer of ________

A

anti- ribonucleoprotein (RNP)

85
Q

IgG4 -related disease is characterized as _________

A

fibro inflammatory

86
Q

Hyperacute rejection is mediated by what type of cell>

A

B cells

87
Q

Chronic Ab mediated recjection will result in ________ of vessels of organ tissue

Acute ab mediated rejection will have a (+) )_________stain

A

Fibrosis

C4d (complement)

88
Q

A person with Sojrogens is more susceptible to what infection?

A

candida yeast of tongue

89
Q

Presence of tubulitis or endothelitis indicates what type of rejection?

A

Acute cellular rejection (t cell mediated )

days, months, years

90
Q

After treatment with immunosuppressive patient is at increased risk for what virus? viral induced tumors?

A

polyomavirus, cytomegalovirus

kaposi sarcoma
lymphoma

91
Q

Indirect pathway of allorecognition is mediated by ________

A

RECIPIENTS antigen presenting cell

92
Q

A 13-year-old boy presents to his pediatrician for a routine visit. He states that he has been in good health with the exception of repeated staphylococcal infections despite adequate treatment. On exam, the pediatrician observes the findings demonstrated in albinism, as well as nystagmus. Which of the following is the most likely mode of inheritance of this patient’s condition?

Describe what a peripheral smear will show?

A

Chediak higashi syndrome

  • failure of phagolysosomal fusion

giant granules

93
Q

What is inheritance of chediak higashi?

A

Autosomal recessive

94
Q

What are 2 disease due to leukocyte disorders?

A

Chediak Higashi+

Chronic granulomatous disease

95
Q

What is the cause of chronic granulomatous disease?

A

failure of phagocyte to produce superoxide

accumulate macrophages because infection persists

96
Q

A 5-year-old female suffers from recurrent infections by Aspergillus species, Pseudomonas species, and Staphylococcus aureus. The patient’s neutrophils are examined in the laboratory . Which of the following is most likely dysfunctional in this patient?

A.Lymphocytes
B.Immunoglobulin class switching
C.Superoxide dismutase
D. Respiratory burst

A

D. respiratory burst

chronic granulomatous diseas

97
Q

Pt with C5-C9 deficiency is more susceptible to ?

A

Neisseria Meningitis

lack of protection against microbes

98
Q

A 47-year-old male presents to the emergency department with facial swelling and trouble breathing. These symptoms began this morning and progressively worsened over the past several hours. Physical examination reveals nonpitting swelling of the face, hands, and arms as well as edema of the tongue and mucus membranes of the mouth and pharynx. The patient reports several episodes of mild facial swelling that occurred during childhood between the ages of 5-18, but he does not recall seeing a physician or receiving treatment for this. This patient most likely has which of the following underlying abnormalities?

A. MHC class I deficiency
B.Defective lysosomal storage proteins
C. Lack of NADPH oxidase
D. Defect in cytoskeletal glycoprotein
E. Deficiency of C1 esterase inhibitor
A

E. C1 inhibitor deficiency

hereditary angioedema
( autosomal dominant)

Very similar to anaphylaxis

99
Q

Corkscrew intestine can be a sxs of ________

A

hereditary angioedema

100
Q

A 3-year-old Cuban-American male has a history of recurrent Pseudomonas and Candida infections. Laboratory analysis reveals no electrolyte abnormalities. Examination of his serum shows decreased levels of IgG and CT scan reveals the absence of a thymus. The child likely has

A. X-linked agammaglobinemia
B.DiGeorge syndrome
C. Severe combined immunodeficiency syndrome
D. Isolated IgA deficiency
E. Common variable immunodeficiency
A

SCID

(Recurrent infections with fungal and bacterial pathogens are likely the result of defective T and B cell immunity. Severe combined immunodeficiency syndrome (SCID) is the only disorder of BOTH B and T cell lineages

101
Q

SCID due to adenosine deaminase deficiency is what type of inheritance?

A

autosomal recessive

102
Q

X linked SCID is due to_______

A

mutation s in IL receptor

inhibits maturation and production of T Cells

103
Q

Failure of pharyngeal pouches 3/ 4 affects develpment of what organs?

A

Di George

thymus
parathyroid
heart / great vessls

104
Q

You are called to examine a one-hour-old baby who was born with a facial defect . The patient’s ears appear to be low-set. On auscultation, there is a holosystolic murmur along the lower left sternal border. Basic labs return notable for hypocalcemia. What is the most likely underlying mechanism responsible for the abnormalities seen in this newborn?

A.X linked agammaglobulinemia
B.Chediak higashi
C.Trinucleotide repeat expansion
D. Partial deletion on chromosome 22

A

D.

105
Q

You are seeing a 4-year-old boy in clinic who is presenting with concern for a primary immune deficiency. He has an unremarkable birth history, but since the age of 6 months he has had recurrent otitis media, bacterial pneumonia, as well as two episodes of sinusitis, and four episodes of conjunctivitis. He has a maternal uncle who died from sepsis secondary to H. influenza pneumonia. If you drew blood work for diagnostic testing, which of the following would you expect to find?

A.Abnormally low number of B cells
B.Abnormally low number of T cells
C. Abnormally high number of B cells
D.Abnormally high number of T cells
E. Elevated immunoglobulin levels
A

Xlinked agamma

risk for infxn increases after maternal ab wane (6 months)

106
Q

Pts with Xlinked agammaglobulinemia have increased risk of infection with __________

A

encapsulated bacterial infection

s. pnuemonia

hemophilus influenzae

s. pyogenes

giardia (protozoa)

107
Q

A 3-month-old is referred to a pediatric immunologist by his pediatrician for further workup of recurrent sinopulmonary infections which have not abated despite adequate treatment. During the workup flow cytometry demonstrates a decrease in normal CD40L cells. Based on these findings, the immunologist decides to pursue a further workup and obtains immunoglobulin levels. Which of the following immunoglobulin profiles is most likely to be observed in this patient?

A.Increased IgE; Decreased IgG, IgM
B.Decreased IgE, IgM, IgA, IgG
C. Increased IgE, IgA; Decreased IgM
D. Increased IgE
E. Increased IgM; Decreased IgG, IgA, IgE
A

E. Increased IgM and decreased IgG, IgA, and IgE is characteristic of hyper-IgM syndrome, which causes severe pyogenic infections in the first months of life.

Hyper IgM syndrome

108
Q

What condition affects B cell class switching?

A

Hyper IgM syndrome

109
Q

30-year-old Caucasian male is brought to the emergency room for recurrent diarrhea. He has had multiple upper respiratory infections since birth and does not take any medications at home. It is determined that Giardia lamblia is responsible for the recurrent diarrhea. The physician performs a serum analysis and finds normal levels of mature B lymphocytes. What other finding on serum analysis predisposes the patient to recurrent diarrheal infections?

A

Low IgG

CVID

The patient has common variable immunodeficiency disorder (CVID), which leads hypoagammaglobulinemia. Specifically, the patient has a deficiency in IgG therefore he has an increased susceptibility to Giardia infection.

110
Q

Deficiency of _______ causes weakened mucosal defenses and increased GI/ urinary bladder infection?

A

Ig A deficiency

111
Q

Chronic diarrhea of CVID is due to ________

A

giardia lamblia

112
Q

6-year-old boy is brought to the emergency department following a car accident in which he suffered multiple injuries. He is accompanied by his mother. She reports that his medical history is notable only for recurrent sinusitis and otitis as a child. He lost a significant amount of blood from the accident, and he is transfused two units of O-negative blood on arrival at the emergency department. Shortly thereafter, he complains of itching and increasing shortness of breath. He develops stridor. Which of the following could have prevented this reaction?

A. Pre-transfusion acetaminophen
B. Pre-transfusion diphenhydramine
C. Administering type-specific blood]
D.Administering washed blood products
E. Administering IVIG with transfusion
A

Ig A deficiency

red cell washing can prevent reactions

113
Q

A 2-month-old boy is brought to his pediatrician for a routine visit. His mother is concerned because he developed eczema one month ago that has not resolved Furthermore, she states that he has seemed to constantly be sick ever since his birth. The pediatrician notes thrombocytopenia. Which of the following laboratory findings is most likely to be observed in this patient?

Increased IgM; Decreased IgG, IgA, IgE
Increased IgE only
Increased IgE, IgA; Decreased IgM
Increased IgE; Decreased IgG, IgM, IgA
Decreased IgE, IgM, IgA, IgG
A

Wiskott aldrich syndrome

114
Q

WHat is the triad of WASP gene mutation

A
  1. thrombocytopenia
  2. eczema
  3. recurrent infections. (t cell loss and hypogammaglobinemia)
115
Q

A mother brings her 10-year-old girl to the pediatrician for a routine visit. During the visit, the girl comments that recently she has been having difficulty with her balance and has been falling more than usual. She also complains of a rash on her face that has not resolved despite trying multiple over-the-counter treatments . Which of the following is the most likely cause of this patient’s symptoms?

Defect in cellular transport
Defect in cell surface receptor
Defect in actin cytoskeleton function
Defect in microtubule function
Defect in DNA repair
A

Defect in DNA repari

Ataxia TElangiectasia

116
Q

Ataxia telangiectasia is what type of inheritance?

A

Auttosomal recessive

(ATM gene mutation- sensor of DNA damage

117
Q

Ataxia telangiectasia involves deficiency of what antibodies?

A

IgA and IgG

118
Q

Waht are the functions of HIV retroviral genes? (4)

A

LTR- initiates transcription

gag- encodes proteins inside virus

env - encodes surface glycoproteins

pol: viral enzymes

119
Q

Which protein is important for drilling into host target cell for HIV

A

gp41

120
Q

What is the function of HIV integrase?

A

inserts proviral DNA into host genome

121
Q

What initiates viral transcription of HIV DNA?

A

releases of NFkB by antigenic stimulation

122
Q

How can HIV access the CNS and cause neuronal damage?

A

Microglia

123
Q

What helps HIV be transported to lymph nodes? what happens in the lymph nodes?

A

mucosal dendritic cells.

viral replication

124
Q

WHat occurs to B cell response to new antigens in HIV?

A

it is IMPAIRED due to reduced T cells

125
Q

____________ (sore throat , muscle aches )occurs at about 3-6 post intial HIV infection.

A

acute HIV syndrome

  • wide dissemination of viurs

seedding lymph organs

126
Q

Does HIV infect neurons fo the brain?

A

NO

pathogenisis is via microglia that secrete cytokines

127
Q

Does HIV infect neurons fo the brain?

A

NO (LO #42)

pathogenisis is via microglia that secrete cytokines

128
Q

Describe the clinical latency period of HIV?

A

This is the 2nd out of 3 stages

it is a period of silent massacre

CD4+ major decline

129
Q

What type of infections is an HIV patient susceptible too in the chronic latent period?

A

Opportunistic

1.pneumocysitis pneumonia

  1. tuberculosis
  2. toxoplasma
  3. candida
  4. cryptosporidiujm
130
Q

Patients with what disease have increased risk of Kaposi sarcom and lymphoma?

A

HIV

131
Q

At about 10 days post infection what is the first detectable substance of HIV?

A

viral RNA NAT

132
Q

What substance is detected years after initial infection?

A

HIV antibody

133
Q

What substance is detected weeks after intial infection

A

antigen p24

134
Q

What accounts for majority of deaths in a patient diagnosed with AIDS?

A

Opportunisitc infection

ex. Myobacterium avium

cryptosporidium (protozoa)

cytomegalovirus

more on pg 253 robbins

135
Q

Give an example of AIDS defining illness?

A

PNeumocystis jiroveci

fungal infection of lungs

136
Q

What causes the virus associated B cell lymphoma in HIV?

A

Uchecked EBV reactivation in latent infected B cells due to CD4+ depletion

137
Q

What 4 lymphomas are implicated with AIDS patients?

A
  1. Hodgkin
  2. CNS
  3. Primary effusino
  4. cervical / anal cancer
138
Q

A 27-year-old man presents to the urgent care clinic with a 2-week history of fever, macular rash, and generalized lymphadenopathy. He denies a sore throat, genital ulcers, or urethral discharge. Sexual history is remarkable for having unprotected sex with both male and female partners while inconsistently using condoms. His last sexual encounter was a month prior to the onset of illness.

What is the classification of his infectious agent?

A

Retroviridae (LEntivirus)

139
Q

What accumulates in AL amyloidosis?

A

immunglobin light chains into the blood

due to mutation

140
Q

Accumulation of SAA protein in AA amyloidosis is due to ________

A

chronic inflammation

causing liver to secrete AA
ex. SLE, osteomylitis, tuberculosis)

141
Q

What is the effect of systemic amyloidosis in the Kidney? Heart ?

A

Kidney: proteinuria and edema

Heart :
dysrhythmias CHF

142
Q

Amyloidosis can cause ___________ of liver, spleen and tongue

A

Enlargement

143
Q

For which of the following patients would you recommend prophylaxis against mycobacterium avium-intracellulare?

A. 22-year old HIV positive female with CD4 count of 750 cells/ microliter and a viral load of 500,000 copies/mL

B. 30-year old HIV positive male with CD4 count of 20 cells/ microliter and a viral load of < 50 copies/mL

C. 45-year old HIV positive female with CD4 count of 250 cells/ microliter and a viral load of 100,000 copies/mL

D. 50-year old HIV positive female with CD4 count of 150 cells/ microliter and a viral load of < 50 copies/mL

5
36-year old HIV positive male with CD4 count of 75 cells/microliter and an undetectable viral load.

A

B.

CD4+ count is the most reliable for TREATMENT

Viral load is not used as an indication for treatment
it can be used to predict progression of HIV

144
Q

A 20-year-old female arrives at the urgent care clinic at her university’s health plan asking for an HIV test. She is an undergraduate at the university and just started having sexual intercourse with her new boyfriend. They use protection only occasionally so she wants to get tested to make sure everything is okay. She has never been tested for STDs before. She reports no symptoms and has not seen a physician regularly for any medical conditions in the past. Her family history is uncertain because she was adopted. Her HIV immunoassay and HIV-1/HIV-2 differentiation immunoassay both come back positive. She asks on the phone, “Doctor, tell it to me straight. Do I have AIDS?” Which of the following is the most accurate response?

A. We have to get a confirmatory PCR test to see if you have AIDS.

B. You do not have AIDS because you just started having sex recently.

C.You have AIDS but this disease is now a manageable condition.

D We need your partner’s information to be sure of your diagnosis.

E. We need additional bloodwork to see if you have AIDS

A

E.
In a patient who is infected with HIV, a diagnosis of AIDS is defined as a CD4+ count less than 200 or affliction with an AIDS-defining illness. Thus additional bloodwork to see if the patients has AIDS would be the most appropriate response.

145
Q

Which of the following would be seen in a patient with deficiency in T and B cells ?

A. Defect in nucleotide excision repair
B. Defect in microtubule function
C. Defect in cell surface receptor
D. Defect in actin cytoskeleton function
E. Defect in cellular transport
A

C.

Xlinked SCID is due to lack of IL receptor

146
Q

Infants with SCID present with?

A

diaper rash , oral candidiasis , FAilure to thrive

147
Q

An 11-year-old boy is brought to the emergency department by his parents with a 2-day history of fever, malaise, and productive cough. On presentation, he is found to be very weak and is having difficulty breathing. His past medical history is significant for multiple prior infections requiring hospitalization including otitis media, upper respiratory infections, pneumonia, and sinusitis. His family history is also significant for a maternal uncle who died of an infection as a child. Lab findings include decreased levels of IgG, IgM, IgA, and plasma cells with normal levels of CD4 positive cells. The protein that is most likely defective in this patient has which of the following functions?

A. Actin polymerization
B. Autoimmune regulation
C. Lysosomal trafficking
D. Nucleotide salvage
E. Protein phosphorylation
A

E. kinase phosphoryates

148
Q

A 2-year-old boy is brought to his pediatrician for evaluation of high fever and malaise. His mother relates that he suffers from frequent infections and has had several episodes of bloody diarrhea in the last year. Physical exam reveals diffuse petechial lesions as well as dry red skin in the elbow and other extremity flexor compartments. His mom remembers that one of her nephews has a similar problem with bleeding and very itchy rashes but cannot recall the details. Which is the patterns of immunoglobulin findings is most likely ?

A

This case is most consistent with a diagnosis of Wiskott-Aldrich syndrome, a disease characterized by the classic triad thrombocytopenia, eczema, and recurrent infections. The disease is caused by a mutation in an actin binding protein and serological findings include

increased IgA and IgE with
low IgM
low IgG.

MOD (13 decks)

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