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COMLEX STEP 1 2017 > Immunology > Flashcards

Immunology Flashcards

1
Q

What is the probably disorder in a young ped pt that: causes very low levels of IgM, IgG, IgA
absence of CD 19+ cells
normal CD4+ and CD56+ cells
bone marrow biopsy showing abnormally high numbers of cells with cytoplasmic mu chains

A

X-linked (Bruton) agammaglobulinemia

-block in the B-cell maturational process

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2
Q

What is the biochemical etiology of X-link Bruton agammaglobinemia

A

mutation in Bruton tyrosine kinase (cytoplasmic tyrosine kinase)

-signalling needed to drive pro-B cells to pre-B cells to mature naive B-cells

the cell is stopped at the point that the heavy chains genes have been rearranged, cells dont produce cytoplasmic mu heavy chains

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3
Q

How is genetic information produced in Herpesviruses

A

Herpesviruses are DNA viruses that use the negative strangd of their genome as a template to transcribe messenger RNA

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4
Q

What are two major chemotactic factors for Neutrophils?

A

C5a

IL-8

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5
Q

What is the likely causal agent in a immunosuppressed pt with ring-enhancing lesions on MRI?

What part of immunity is likely deficienct in this patient?

A

Cerebral toxoplasmosis

T-helper 1 cells (toxoplasmosis is intracellular)

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6
Q

What disease is characterized by absence of CD18 antigens?

A

Leukocyte adhesion deficiency (LAD) - autosomal recessive disorder

–CD18 are needed to form integrins = essential for leukocyte adhesion to endothelial surfaces and migration to peripheral tissues

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7
Q

Which cell types are predominantly responsible for IFN-gamma production?

A

T-lymphocytes and natural killer cells

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8
Q

What is the mechanism of eosinophilia during parasitic infections

A

Eosinophil proliferation is caused by IL-5 produced by TH2 and Mast cells

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9
Q

what are the three chemotactic factors for neutrophils?

A

Leukotriene B4
IL-8
C5a

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10
Q

What is the disease and what is the deficient protein?:

  • low blood T lymphocyte count
  • severely decreased serum immunoglobulin level
A

SCID

-deficienty Adenosine deaminase

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11
Q

What immune cell(s) are activated by IL-2

A

T-cells (CD4, CD8)
B-cells
NK cells
Monocytes

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12
Q

What is the disease:

infant with recurrent bacterial, viral, fungal, and opportunistic infections + failure to thrive, chronic diarrhea

A

SCID

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13
Q

What two cells are involved in an antigen skin test?

A

CD4 T cells

Macrophages

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14
Q

What type of hypersensitivity is a hyperacute rejection of a transplant?

A

Type II hypersensitivity - preformed Abs in circulation against graft

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15
Q

Which cytokines do the following to TH1 cells?

  1. stimulates
  2. inhibits
A
  1. IFN-gamma, IL-12

2. IL-4, IL-10

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16
Q

Which cytokines do the following to TH2 cells?

  1. stimulates
  2. inhibits
A
  1. IL-4

2. IFN-gamma

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17
Q

What cytokines are responsible for
1. formation
2. maintenance
of granulomas

A
  1. TNF-alpha

2. INF-gamma

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18
Q

What type of hypersensitivity is acute hemolytic transfusion reaction?
what the mechanism of hemolysis?

A 
Type II (Ab mediated)
-complement-mediated cell lysis
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19
Q

What do the following Sx suggest?
Bilateral hilar adenopathy
elevated serum Calcium
elevated serum Angiotensin-converting enzyme

A

Sarcoidosis - formation of noncaseating granulomas

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20
Q

What are fluid findings in bronchoalveolar lavage fluid of sarcoidosis

A

predominance of CD4 T cells

CD4:CD8 ratio > 2:1

21
Q

Which cytokine is responsible for pus accumulation in an injury

A 
pus = primarily neutrophils
IL-8 = neutrophil chemotaxis
22
Q

What is result of a defect in CD40 ligand - CD40 interaction? Sx?

A

Sx - recurrent sinopulmonary, gastrointestinal, opportunistic infections

Hyperimmunoglobulin M (no class switching)

23
Q

what two cell markers prevent complement activation on self-cells

what happens when there is a deficiency in these markers

A

CD59, CD55(decay accelerating factor

paroxysmal nocturnal hemoglobinuria

24
Q

what is the associated disease?

Anti-Jo-1, Anti-SRP, anti-Mi-2

A

Polymyositis, dermatomyositis

25
Q

what is the associated disease?

Antiphospholipase A2 receptor

A

primary Membranous Nephropathy

26
Q

what is the associated disease?

Anti-Scl-70 (Anti-DNA topoisomerase I)

A

Diffuse Scleroderma

27
Q

what is the associated disease?

Anti-smooth muscle

A

Autoimmune hepatitis type I

28
Q

what is the associated disease?

Anti-U1 RNP (ribonucleoprotein)

A

Mixed Connective tissue disease

29
Q

what is the associated disease?
MPO-ANCA
p-ANCA

A

Microscopic polyangiitis

eosinophilic granulomatosis w polyangiitis (Churg-Strauss Syndrome)

30
Q

what is the associated disease?
PR3-ANCA
c-ANCA

A

Granulomatosis with polyangiitis (Wegener)

31
Q 
What is the disease and genetic defect:
Ataxia - cerebellar atrophy
spider Angiomas
IgA, IgG, IgE deficiency
increased AFP
A

Ataxia-telangiectasia

defects in ATM gene (involved in DNA double strand breaks)

32
Q

What substance is released by mitochondria to induce indirect pathway of apoptosis?

A

cytochrome c

33
Q

What is the most important source of protection against the influenza virus?

A

humoral antibodies against Hemagglutinin

34
Q

What part of the immune system is responsible for protecting against

  1. superficial candidal infection
  2. hematogenous candidal infection
A
  1. T lymphocyte

2. Neutrophils

35
Q

what deficiency is involved with disseminated mycobacterial disease in infancy or early childhood

A

inherited defects involving interferon-gamma signalling

36
Q

What cell is Programmed death (PD-1) Receptor located on?

what is its function?

A

located on cytotoxic T-cells
PD-1 receptor activation downregulates cytotoxic capabilities

some cancers increase PD-1 ligand expression on their cells

37
Q

identify:
punch biopsy of epidermis
stellar cells w intracytoplasmic granules of a tennis raquet shape
cells have some myeloid surface markers and can interact with T lymphocytes

A

Langerhans cells

38
Q

Which bacteria produces IgA proteases (increase mucosal adherence)

A

Strep Pneumo
Haemophilus Influenzae
Neisseria Gono, Meningitidis

39
Q

what does the CD14 marker indicate?

A

Monocyte-Macrophage cell lineage

40
Q

Toxic shock syndrome usually involve which bacteria (tampons/nasal packing)
and which immune cells?

A

S. aureus
Macrophages + T-lymphocytes
(widespread activation of T-lymphocytes)

41
Q

PSGN is what type of hypersensitivity?

42
Q

Identify dz and deficiency enzyme:

loss of early motor skills
hepatosplenomegaly
mental retardation
cherry-red macular spot

what other dz can present this way, how can you tell the difference?

A

Niemann-Pick
-sphingomyelinase deficiency

Tay-Sachs can present like this but DOES NOT INVOLVE THE LIVER

43
Q

What recombinate cytokine could be used to treat MS? why?

A

B-interferon - anti-inflammatory effects that suppress the pro-inflammatory cytokines that produced in MS

44
Q

What is the type of hypersensitive and name of phenomenon:

2 month old w rash. pt was seen 6 hrs earlier and given vaccinations: DTaP (the diptheria, tetanus, pertussis vaccination). Irritation present at injection site, edematous lesion with induration and necrotic edges. baby is crying alot

A

Type III - Arthus reaction
–occurs after vaccination with tetanus or diphtheria

–presentation = severe pain, swelling, edema, lesion can become necrotic

45
Q

10 year old w ear ache. hx or recurrent ear infections, hx of pneumonia, meningitis infections. outbreaks of red patches and bumps on skin since infancy. Febrile, erythematous edematous tympanic membrane w purulent discharge on the right side. skin has several purpuric patches on the lower extremities.

what is dz, inheritance pattern, and defective immune fxn?

A

Wiskott-Aldrich Syndrome (WAS)

  • X-linked
  • low IgM levels, elevated IgA, IgE
  • thrombocytopenia, cellular, and humoral deficiency, often w hx of purulent infections ENCAPSULATED bacteria
46
Q

11 year old w fever, and right hand pain/redness. his of recurrent staphylococcal infections. Deciduous teeth. Deep-set eyes w broad nose. Erythematous patches w vesicular and flaking pruritic areas.

what is dz, inheritance, etiology?

A

Job syndrome (hyper IgE)

  • -autosomal dominant
  • -recurrent staphylococcus, candida infections
47
Q

What is causing Sx:
18 year old w chest pain that increases in intensity upon inspiration. Physical exam shows presence of multiple buccal ulcers and thin fragile hair. Lab analysis shows positive plasma reagin test.

A

Systemic lupus erythematosus

  • -oropharyngeal ulcers
  • -pericarditis
  • -alopecia
  • FALSE POSITIVE RPR or VDRL tests due to cross reactivity with cardiolipin
48
Q

21 yr old female with hx of severe bacterial meningitis w rash treated w ceftriaxone 6 months ago presents with pain in wrists and ankles and sore throat. Fever and macular rash with ulcerations over wrists and ankles

  1. what is dz
  2. what immune deficiency does she have?
A

Disseminated gonorrhea infection by Neisseria gonorrhoeae

—C5-C9 complement deficiency
= cant form membrane attack complex
= high risk of Neisseria infection

COMLEX STEP 1 2017 (19 decks)

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