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KAPLAN FLASH CARDS > Immunology > Flashcards

Immunology Flashcards

1
Q

Inheritance of Wiskott-Aldrich Syndrome (WAS) and antibody findings

A

X-linked recessive

  • Elevated IgA & IgE
  • IgM low

**WASP protein normally allows cytoskeleton rearrangements for antigen binding, but this is dysfunctional and T cells under-respond

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2
Q

Characteristic signs of WAS (Wiskott-Aldrich syndrome)

A
  • Thrombocytopenia w/ hepatosplenomegaly
  • Eczema
  • Immunodeficiency (recurrent infections: respiratory & skin)

*The problem is lymphocytes are dysfunctional due to WASP protein

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3
Q

Who does WAS (Wiskott-Aldrich syndrome) effect?

A

Young boys

*Usually die by 6 from infection

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4
Q

What malignancy is WAS (Wiskott-Aldrich syndrome) inclined to develop?

A

Lymphoid

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5
Q

Treatment & cure of WAS (Wiskott-Aldrich syndrome)

A

Bone marrow transplant from HLA identical sibling

*complete reversal of platelet & immune defect

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6
Q

Cellular immune responses are promoted by what?

A

CD4+ and Th1 cells

Secrete IFN-gamma & IL-12 to activate macrophages & CD8+ T cells

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7
Q

Which interleukins does Th2 secrete and what do they do?

A

IL-4, IL-5, IL-6

Induce B cell class switching and promote humoral immune response

IL4: IgE
IL5: IgA
IL6: Acute inflammation

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8
Q

Function of Th17

A

Induce pro-inflammatory cytokines that recruit & promote neutrophil responses against extracellular pathogens

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9
Q

Purpose of IL-7 and mutation of its gamma chain causes what?

A

PURPOSE: Causes pluripotent stem cells to differentiate down the T-cell lineage during hematopoiesis

DISEASE: SCID [severe combined immunodeficiency]

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10
Q

Phenotypically, what is demonstrated in patients with SCID?

A

Absent CD3 (T cell)

Present CD19 (B cell)

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11
Q

Phenotypically, what is demonstrated in patients with adenosine deaminase deficiency?

A

Absence of CD3 & CD19

*Accumulated metabolites due to deficiency of adenosine deaminase causes toxins metabolites to accumulate in lymphocytes, killing them

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12
Q

What is defective in hyper-IgM syndrome

A

CD40L

*Impossible for B cells to receive the signal to isotype switch

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13
Q

Phenotypically, what is demonstrated in patients with RAG gene mutation?

A

VDJ recombination can’t occur, thus no CD3 or CD19

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14
Q

Defective development of what (embryologically) causes DiGeorge syndrome?

A

3rd & 4th pharyngeal pouch

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15
Q

What chromosomal abnormality causes DiGeorge syndrome?

A

Deletion at 22q11.2

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16
Q

What CD markers are present on mature T lymphocytes?

A

CD3 & CD5

*In DiGeorge syndrome, they are reduced due to thyme hypoplasia/aplasia

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17
Q

What CD markers are present on NKCs?

A

CD16 & CD56

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18
Q

What CD markers are present on mature B lymphocytes?

A

CD19 & CD20

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19
Q

What is defective in ataxia-telangiectasia and what are the consequences?

A

ATM gene mutation causing impaired VDJ recombination

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20
Q

What condition is associated with c-ANCA

A

Granulomatosis with polyangiitis

*Formerly called Wegener granulomatosis

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21
Q

What condition is associated with p-ANCA

A

Churg-Strauss syndrome

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22
Q

2 key characteristics always seen together in c-ANCA condition

A

Granulomatosis with polyangiitis:

1) Necrotizing vasculitis of the upper & lower respiratory tract –> nasal septal perforation, sinus pain, cough/hemoptysis
2) Kidney: crescentic, rapidly progressive GN

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23
Q

Key characteristics in p-ANCA condition

A

Asthma

Eosinophilia

*Eosinophil-rich necrotizing vasculitis; systemic effects

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24
Q

Most important CHEMOTACTIC factors for neutrophils

A

C5a & IL-8

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25
Q

Toxoid vaccine examples

A

Diptheria
Tetanus

*Chemically inactivated

26
Q

Attenuated vaccine examples

A

*Weakened virus

“Attention Teachers! Please Vaccinate Small, Beautiful Young Infants with MMR Regularly”

  • Adenovirus
  • Typhoid
  • Polio (Sabin)
  • Varicella
  • Smallpox
  • BCG
  • Yellow fever
  • Influenza (intranasal)
  • MMR
  • Rotavirus
27
Q

Killed, whole-cell vaccine examples

A

“A TRIP could Kill you”

  • Hepatitis A
  • Typhoid
  • Rabies
  • Influenza
  • Polio (SalK)
28
Q

Example of toxoid + polyribitol phosphate vaccine

A

Haemophilus influenza type B

*The capsule (polyribitol phosphate) alone would only produce a weak IgM response (T-cell independent)

**Must conjugate capsule to a toxoid (diphtheria toxoid) to induce memory (T-cell dependent)

29
Q

Cause of Lambert-Eaton Myasthenic Syndrome (LEMS)

A

Autoimmune neuromuscular disorder due to antibodies against presynaptic calcium channels

30
Q

Symptoms of LEMS

A
  • Proximal muscle weakness

- Decreased tendon reflex (improving with prolonged muscle contraction)

31
Q

What is often associated with LEMS

A

SCLC

32
Q

What cytokines are overproduced in a super antigen infection & why?

A

T CELL:
IFN-gamma

MACROPHAGE:
IL-1
IL-6
TNF-alpha

*Superantigen crosslinks MHC II (macrophage) with TCR, inducing huge cytokine overproduction

33
Q

What organisms are splenectomized patients most at risk of infection from?

A

Gram +, catalase -

  • S. pneumonia
  • *Hib
  • **N. meningitides
34
Q

What organisms are patients with NADPH oxidase gene defect at risk for? Give examples

A

Catalase + infections

  • S. aureus
  • Aspergillus
  • Nocardia
  • Salmonella
  • Candida

*They neutralize H2O2 quickly into water and oxygen

35
Q

Subunit Vaccine examples

A
  • HBV (HBsAg)
  • HPV (6,11,16,18)
  • N. meningitidis
  • S. pneumonia (13,23)
  • Hib
36
Q

Cause & symptoms of LAD (leukocyte adhesion deficiency)

A

CD18 defect

  • Delayed umbilical cord separation
  • Omphalitis
  • Recurrent bacterial infections
37
Q

Common lab finding in LAD (leukocyte adhesion deficiency)

A

Increased neutrophils in the blood

*Neutrophils can’t migrate to sites of inflammation correctly and remain in the blood

38
Q

Which hypersensitivity reaction is Goodpasture Syndrome

A

Type II, cytotoxic

39
Q

In TSS (toxic shock syndrome), what cytokines are released and from what cells?

A

Macrophages: IL-1, IL-6, TNF-alpha

TH1: IFN-gamma

40
Q

Characteristics of IgA deficiency

A
  • Increased mucosal pathogens
  • Atopic allergy (High IgE)
  • Transfusion reaction
41
Q

Why is IgE higher in IgA deficiency?

A

Increased isotope switching due to their inability to produce IgA

*anti-IgA & IgE antibodies

**Patients can only receive blood products from other IgA deficient donors

42
Q

When do we use ELISA

A

Measure antibody titers against viruses & other pathogens

43
Q

When do we use Northern blots?

A

Determine RNA levels in a cell

44
Q

When do we use Southern blots

A

Study genomic DNA

45
Q

When do we use subtractive hybridization?

A

Research technique, not clinical assay

*Subtracts out the RNA of a closely related cell type in order to see the RNA from genes that are transcribed by a specific cell type

46
Q

How can we calculate amount of globulin in blood?

A

Total protein - albumin = globulins

47
Q

Hallmark of LAD (leukocyte adhesion deficiency)

A
  • Inability to form pus or abscesses, recurrent bacterial infections, severely delayed wound healing
  • Delayed umbilical cord separation in newborns
  • Neutrophilia (5-10x higher) due to inability of cells to extravasate into tissues
48
Q

When do we use the microcytotoxicity test?

A

To determine HLA class I expression of recipient & potential donor cells in order to select a compatible match

49
Q

When do we use a mixed lymphocyte reaction test?

A

Determine compatibility of transplant recipient and donor with regard to HLA class II molecules

**Microcytotoxicity test is for HLA class I

50
Q

Cytotoxic VS noncytotoxic type II hypersensitivity reaction

A

CYTOTOXIC:
Antibodies binding to cells or tissues and causing death of the cells or tissues
ie) Mismatch blood transfusion

NONCYTOTOXIC:
Antibodies binding to cells or tissues and changing their function
ie) Graves or Myasthenia Gravis

51
Q

Arthus Reaction

A

Varian of type III hypersensitivity in which local vasculitis induces tissue necrosis, usually of the skin

52
Q

What deficiency is seen in Bruton agammaglobulinemia?

A

Bruton Tyrosine Kinase

*impaired B cell maturation from pre-B cell to mature B cel, causing a total absence of any circulating B cells (CD19)

53
Q

When do symptoms present in x-linked agammaglobulinemia?

A

6 months, after which maternal antibodies are no longer active

54
Q

What are XLA (x-linked agammaglobulinemia) patients susceptible to?

A

S. pneumonia
Hib
Pseudomonas spp

Sx:
Sepsis
Meningitis
Osteomyelitis

55
Q

Infants with XLA will present with what?

A

Recurrent otitis media
Sinopulmonary infections
Pneumonia

56
Q

Adults with XLA will present with what?

A

Skin infections caused by staphylococcus and group a streptococcus

Chronic upper & lower respiratory infections

57
Q

Define allotype

A

Minor amino acid differences in the constant domains of antibody molecules, dictated by genetic inheritance

ie) Paternity test

58
Q

Define idiotype

A

Antigen-recognition portions of immunoglobulin molecule specific to unique antigens we have been exposed to

*EVERY antibody has a different idiotype

59
Q

Define isotype

A

5 classes of immunoglobulins:

IgG, IgA, IgM, IgE, IgD

60
Q

Symptoms associated with hyper-IgM syndrome

A

Recurrent sinopulmonary infections

  • Pneumonia
  • Sinusitis
  • Otitis Media

KAPLAN FLASH CARDS (10 decks)

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