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Step 1 Buzzwords > Buzzwords 1 > Flashcards

Buzzwords 1 Flashcards

1
Q

Increased synthesis of disorganized collagen (types 1 and 3)

A

Keloid formation

Primary pathogenesis

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2
Q

Increased synthesis of parallel (non-disorganized) type 3 collagen

A

Hypertrophic scar formation

Primary pathogenesis

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3
Q

Deficient AIRE (autoimmune regulator)

A

Autoimmune polyendocrine syndrome 1

Pathogenesis

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4
Q

Expresses tissue-restricted self-antigens in the thymic medulla for negative selection

A

AIRE

Normal function

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5
Q

Fas mutation leads to impaired negative selection in the thymic medulla and increased self-reacting lymphocytes

A

Autoimmune lymphoproliferative syndrome

Pathogenesis

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6
Q

Deficient FOXP3 (normally regulates Tregs)

A

IPEX

Pathogenesis

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7
Q

Increased risk of severe, recurrent pyogenic sinus and respiratory infections, especially with encapsulated organisms

A

C1-C4 (early complement) deficiencies

Presentation

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8
Q

Increased susceptibility to recurrent Neisseria bacteremia

A

C5-C9 (late complement) deficiency

Presentation

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9
Q

Prevents complement activation on self-cells

A

C1 esterase inhibitor

Function

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10
Q

Hereditary angioedema

A

C1 esterase inhibitor deficiency

Presentation

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11
Q

Defect in the PIGA gene

A

CD55 (DAF) deficiency

Pathogenesis

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12
Q

Paroxysmal nocturnal hemoglobinuria

A

CD55 (DAF) deficiency

Presentation

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13
Q

Most common primary immunodeficiency

A

Selective IgA deficiency

Superlative

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14
Q

Defect in B-cell DIFFERENTIATION

A

CVID

Pathogenesis

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15
Q

Defect in B-cell MATURATION

A

Bruton agammaglobulinemia

Pathogenesis

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16
Q

Recurrent bacterial (especially encapsulated) and enteroviral infections PRESENTING AFTER 6 MONTHS

A

Bruton agammaglobulinemia

Presentation

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17
Q

Increased susceptibility to Giardia

A

Selective IgA deficiency

Increase susceptibility to what organism?

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18
Q

Increased risk of AI disease, bronchiectasis, lymphoma, sinopulmonary infections USUALLY PRESENTING AFTER AGE 2

A

CVID

Presentation

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19
Q

Absent B cells in peripheral blood and absent/scanty lymph nodes and tonsils

A

Bruton agammaglobulinemia

Key lab finding

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20
Q

Decreased plasma cells

A

CVID

Key lab finding

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21
Q

Deletion of 22q11 leads to aberrant/failed development of third and fourth pharyngeal pouches -> absent (or hypoplastic) thymus and parathyroid glands

A

Thymic aplasia (DiGeroge syndrome)

Pathogenesis

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22
Q

Decreased Th1 response due to a specific IL-receptor deficiency

A

IL-12 receptor deficiency

Pathogenesis

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23
Q

STAT3 mutation -> deficiency of Th17 cells -> impaired recruitment of neutrophils to infection sites

A

Hyper Ig-E syndrome (Job syndrome)

Pathogenesis

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24
Q

Presents after BCG vaccination

A

IL-12 receptor deficiency

Presents after what inciting event?

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25
Q

Non-inflammed staphylococcal abscesses

A

Hyper-IgE (Job syndrome)

Unique presenting symptom

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26
Q

Retained primary teeth

A

Hyper-IgE (Job syndrome)

Unique presenting symptom

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27
Q

Which immunodeficiency requires lifelong antimycobacterial antibiotics?

A

IL-12 receptor deficiency

Requires treatment with lifelong ___

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28
Q

Adenosine deaminase deficiency

A

SCID

Autosomal recessive cause

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29
Q

Defective IL-2R gamma chain

A

SCID

X-linked recessive cause

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30
Q

Defective ATM gene -> failure to detect DNA damage -> failure to halt cell cycle -> accumulation of mutations

A

Ataxia-telangiectasia

Pathogenesis

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31
Q

Defect in CD40L on Th cells -> class-switching defect

A

Hyper-IgM syndrome

Pathogenesis

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32
Q

Mutated WASp gene -> abnormal cytoskeleton function in leukocytes and platelets -> defective antigen presentation

A

Wiskott-Aldrich syndrome

Pathogenesis

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33
Q

DDx - absent or hypoplastic thymus

A

DiGeorge syndrome
SCID

(Both present with what symptom?)

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34
Q

Deficient ADA -> increased dATP -> toxic to lymphocytes

A

ADA deficiency ( autosomal recessive cause of SCID)

Pathogenesis

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35
Q

Cytokines that mediate cachexia secondary to chronic diseases like cancer, AIDS, HF, COPD, etc.

A

TNF-alpha, IL-1, IL-6, IFN-gamma

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36
Q

Defective LFA-1 integrin (CD18) protein on phagocytes -> impaired phagocyte migration and chemotaxis

A

Leukocyte adhesion deficiency type 1

Pathogenesis

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37
Q

Decreased Sialyl-Lewis ligands -> impaired margination and rolling of leukocytes

A

Leukocyte adhesion deficiency type 2

Pathogenesis

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38
Q

Impaired beta-3 integrins on platelets

A

Leukocyte adhesion deficiency type 3

Pathogenesis

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39
Q

Absent pus

A

Leukocyte adhesion deficiency type 1

Unique presenting symptom

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40
Q

Which type of leukocyte adhesion deficiency does not present with delayed separation of the umbilical cord?

A

Leukocyte adhesion deficiency type 2; milder, less severe and fewer infections

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41
Q

Increased neutrophils in the blood + absence of neutrophils at infection sites

A

Leukocyte adhesion deficiency type 1

Key lab finding

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42
Q

Defective LYST gene -> microtubule dysfunction in neutrophil phagosome-lysosome fusion

A

Chediak-Higashi syndrome

Pathogenesis

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43
Q

Giant granules in granulocytes and platelets (blood smear)

A

Chediak-Higashi syndrome

Blood smear finding

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44
Q

Partial oculocutaneous albinism

A

Chediak-Higashi syndrome

Unique presenting symptom

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45
Q

Defective NADPH oxidase -> decreased generation of ROS in phagolyosomes -> decreased respiratory burst in neutrophils -> decreased intracellular killing

A

Chronic granulomatous disease

Pathogenesis

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46
Q

Abnormal dihydrorhodamine test (decreased green fluorescence)

A

Chronic granulomatous disease

(Diagnostic test)

*This test measures neutrophil superoxide production

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47
Q

Ataxia, telangiectasia (spider angioma), IgA deficiency

A

Ataxia-telangiectasia

Triad

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48
Q

Thrombocytopenia, eczema, recurrent pyogenic infections that WORSEN WITH AGE

A

Wiskott-Aldrich syndrome

Presenting symptoms

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49
Q

NO T-cells on flow cytometry

A

SCID

Key lab finding

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50
Q

Increased AFP (+ decreased IgA, IgE, IgG)

A

Ataxia-telangiectasis

Key lab finding

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51
Q

Increased (or normal) IgM (+very decreased IgA, IgE, IgG)

A

Hyper-IgM syndrome

Key lab finding

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52
Q

Increased IgA, IgE (+normal or decreased IgG, IgM)

A

Wiskott-Aldrich syndrome

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53
Q

Decreased T-cell receptor excision circles (TRECs)

A

SCID

Key lab finding

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54
Q

Curative treatment for SCID?

A

Bone marrow transplant

Curative treatment for which immunodeficiency?

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55
Q

Recurrent bacterial and fungal infections of the lungs, skin, lymph nodes, and liver, especially with CATALASE POSITIVE ORGANISMS

A

Chronic granulomatous disease

Presentation

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56
Q

SEVERE recurrent infections with bacteria, fungi, viruses, protozoa; chronic diarrhea, thrush, and failure to thrive

A

SCID

Presentation

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57
Q

Severe recurrent pyogenic sinopulmonary and GI infections “early,” including OPPORTUNISTIC INFECTIONS WITH PJP, CRYPTOSPORIDIUM, AND CMV

A

Hyper-IgM syndrome

Presentation

58
Q

Which 2 immunodeficiencies involve recurrent bacterial infections with encapsulated bacteria?

A

C1-C4 complement deficiency (pyogenic sinopulmonary)

Bruton agammaglobulinemia (also see enterovirus recurrence)

(Shared feature?)

59
Q

Recurrent disseminated mycobacterial infection (and fungal infection)?

A

IL-12 receptor deficiency

Presentation

60
Q

Recurrent skin and mucous membrane infection with Candida albicans

A

Chronic mucocutaneous candidiasis

(Presentation)

Remember, this is a T-cell deficiency

61
Q

T-cells are affected in which 2 immunodeficiencies? What happens to them?

A

DiGeorge syndrome - decrease in mature T cells

SCID - absent T cells

62
Q

B-cells are affected in which 2 immunodeficiencies? What happens to them?

A

Bruton - no B cells in the periphery

CVID - decreased plasma cells

63
Q

Which two immunodeficiencies involve a decrease in IFN-gamma?

A

IL-12 receptor deficiency
Hyper-IgE syndrome (also has increased IgE and eosinophils)

(Shared feature?)

64
Q

DDx - absent germinal centers in lymph nodes (3)

A

SCID
Bruton agammaglobulinemia
Hyper IgM syndrome

(Shared feature?)

65
Q

Chediak-Higashi syndrome is most commonly diagnosed in ___.

A

Childhood

Which immunodeficiency is most commonly diagnosed in childhood?

66
Q

Avoid live vaccination in these two immunodeficiencies

A

Bruton
SCID

(Shared feature?)

67
Q

Lymph node cluster draining the head and neck?

A

Cervical

Drains?

68
Q

Lymph node cluster draining the dorsolateral foot and posterior calf?

A

Popliteal

Drains?

69
Q

Lymph node cluster draining the trachea and esophagus?

A

Mediastinal

Drains?

70
Q

Lymph node cluster draining the anal canal below the pectinate line, skin below the umbilicus except the popliteal area, scrotum, and vulva

A

Superficial inguinal

Drains?

71
Q

Lymph node cluster draining the lungs

A

Hilar

Drains?

72
Q

Lymph node cluster draining the lower rectum to the anal canal above the pectinate line, bladder, middle 1/3 of vagina, cervix, prostate

A

Internal iliac

Drains?

73
Q

Lymph node cluster draining the upper limb, breast, skin above the umbilicus?

A

Axillary

Drains?

74
Q

Lymph node cluster draining the testes, ovaries, kidneys, uterus?

A

Para-aortic

Drains?

75
Q

Lymph node cluster draining the liver, spleen, pancreas, stomach, upper duodenum?

A

Celiac

Drains?

76
Q

Lymph node cluster draining the lower duodenum, jejunum, ileum, colon to the splenic flexure?

A

Superior mesenteric

Drains?

77
Q

Lymph node cluster draining the colon from the splenic flexure to the upper rectum?

A

Inferior mesenteric

Drains?

78
Q

What lymph node cluster is a primary lung cancer associated with?

A

Primary lung cancer

79
Q

Which two lymph node clusters are associated with granulomatous disease?

A

Mediastinal and hilar

80
Q

What lymph node cluster drains the skin above the umbilicus? Below the umbilicus?

A

Above - axillary

Below - superficial inguinal (except the popliteal area)

81
Q

Track drainage to lymph node clusters through the GI tract, beginning with the esophagus.

A

Esophagus - Mediastinal

Stomach - Celiac

Small intestine - Celiac (upper duodenum), Superior mesenteric (lower duodenum through the ileum)

Large intestine - Superior mesenteric (colon to the splenic flexure), Inferior mesenteric (colon from the splenic flexure to the upper rectum)

Rectum - Inferior mesenteric (upper), Internal iliac (lower rectum to the anal canal, aka above the pectinate line), Superficial inguinal (anal canal below the pectinate line)

82
Q

Which lymph node cluster drains the gonads?

A

Para-aortic (testes, ovaries, uterus)

83
Q

Compare the superficial and deep lymphatic drainage of the lower extremities.

A

Superficial - follow venous system, drain directly to the superficial inguinal nodes via long saphenous vein (medial extremities)

Deep - follow arterial system, drain into popliteal, then inguinal nodes (lateral extremities)

84
Q

Explain lymph node drainage of the reproductive organs.

A

Para-aortic - testes, ovaries, uterus
Internal iliac - vagina (middle 1/3), cervix, prostate
Superficial inguinal - scrotum, vulva
Deep inguinal - glans penis, penile urethra

85
Q

Remove encapsulated bacteria

A

Splenic macrophages

Unique role

86
Q

Which cell does not express MHC I?

A

RBCs

87
Q

Structure of MHC I?

A

1 long chain (A1, A2, A3)

1 short chain (B2)

88
Q

Structure of MHC II?

A

2 equal chains (A1, A2, B1, B2)

89
Q

HLA-A3? (1)

A

Hemochromatosis

HLA subtype

90
Q

HLA-B8 (3?)

A

Addison disease
Myasthenia gravis
Graves disease

(HLA subtype)

91
Q

Mnemonic for HLA-B8?

A

Don’t B late(8), Dr. ADDISON, or else you’ll send MY patient to the GRAVE

(Addison disease, Myasthenia gravis, Graves disease)

92
Q

HLA-B27?

A

Psoriatic arthritis
Ankylosing spondylitis
IBD-associated arthritis
Reactive arthritis

(HLA subtype)

93
Q

HLA-DQ2/DQ8?

A

Celiac disease

HLA subtype

94
Q

HLA-DR2?

A

MS, Hay fever, SLE, Goodpasture syndrome

95
Q

HLA-DR3?

A

DM1, SLE, Graves disease, Hashimoto thyroiditis, Addison disease

(HLA subtype?)

96
Q

HLA-DR4?

A

RA, DM1, Addison disease

HLA subtype?

97
Q

HLA-DR5?

A

Hashimoto thyroiditis

HLA subtype?

98
Q

Addison disease - HLA subtypes

A

B8, DR3, DR4

99
Q

Graves disease - HLA subtypes

A

B8, DR3

100
Q

SLE - HLA subtypes

A

DR2, DR3

101
Q

Hashimoto thyroiditis - HLA subtypes

A

DR3, 5

“Hashimoto is an ODD DoctoR”

102
Q

DM1 - HLA subtypes?

A

DR3, 4

103
Q

What activates an NK cell to kill? (3 major things)

A
  1. Non-specific activation signal on target
  2. ABSENCE of MHC 1 on target
  3. CD16 (on NK) binds Fc region of bound Ig
104
Q

Enteropathy, endocrinopathy, nail dystrophy, dermatitis, other AI dermatologic conditions

A

IPEX

General symptoms

105
Q

Associated with DM in male infants

A

IPEX

106
Q

2 cell signals required for T-cell activation?

A
  1. MHC II (w/Ag) + TCR (CD4) or MHC I (w/Ag) + TCR (CD8)

2. Costimulatory B7 protein (CD80/86) + CD28 (T cell)

107
Q

2 cell signals required for B-cell activation?

A
  1. TCR (activated Th cell) + MHC II (w/Ag)

2. CD40L (Th cell) + CD40 (B cell)

108
Q

Ab in breast millk?

A

IgA

109
Q

Role of C3b?

A

Opsonization (also IgG)

110
Q

Roles of C3a, C4a, C5a?

A

Anaphylaxis

111
Q

Roles of C5b-9?

A

Cytolysis by MAC

112
Q

Fever and sepsis?

A

IL-1, IL-6, TNF-alpha

113
Q

Maintains granulomas in TB?

A

TNF-alpha

114
Q

Cause of green sputum?

A

Myeloperoxidase (blue-green heme-containing pigment)

115
Q

NK cells:
Activated by?
Secrete?

A

Activated by IL2, IL12, IFN-alpha, IFN-beta

Secrete IFN-gamma

116
Q

Th1 cells:
Activated by?
Inhibited by?
Secrete?

A

Activated by IL-12, IFN-gamma
Inhibited by IL-4, IL-10
Secrete IFN-gamma

117
Q

Th2 cells:
Activated by?
Inhibited by?
Secrete?

A

Activated by IL-2, IL-4
Inhibited by IFN-gamma
Secreted IL-4, 5, 6, 10, 13

118
Q

Th17 cells:
Activated by?
Inhibited by?
Secrete?

A

Activated by IL1, IL-6, TGF-beta
Inhibited by: IL-4, IFN-gamma
Secrete IL-17, IL-21, IL-22

119
Q

Treg cells:
Activated by?
Inhibited by?
Secrete?

A

Activated by IL-2, TGF-beta
Inhibited by IL-6
Secrete IL-10, IL-35, TGF-beta

120
Q

Macrophages secrete?

A

IL-1, IL-6, IL-8, IL-12, INF-alpha

121
Q

What do all T-cells secrete?

A

IL-2, IL3

122
Q

Purpose of IL-17, 21, 22?

A

Activate neutrophils to attack extracellular microbes

123
Q

Stimulates production of acute phase proteins

A

IL-6

124
Q

Stimualtes vascular leak?

A

TNF-alpha

125
Q

Class switching to IgG and IgE?

A

IL-4

126
Q

Class switching to IgA?

A

IL-5

127
Q

CD25?

A

Treg

128
Q

CD19, CD20, CD21, CD40?

A

B cells

129
Q

Macrophage CDs?

A

CD14 (PAMP receptor), CD40, CCR5, MHC II, B7 (CD 80/86), Fc and C3b receptors

130
Q

NK cell CDs?

A

CD16, CD56

131
Q

Hematopoietic stem cells CD?

A

CD34

132
Q

Passive vs. active - anti-toxin vs. toxoid?

A

Passive - anti-toxin

Active - toxoid

133
Q

When are patients given preformed antibodies (passive)?

A

After exposure to Tetanus toxin, Botulinum toxin, HBV, Varicella, Rabies virus, or diphtheria toxin

“To Be Healed Very Rapidly or Die”

134
Q

When are patients given combined passive and active immunization?

A

Hepatits B and Rabies

135
Q

2 toxoid vaccines?

A

C. tetani

C. diphtheriae

136
Q

Ab-mediated HSN rxns? Which Ab?

A

I - IgE
II - IgG/IgM
III - Ag-Ab complexes (mostly IgG)

137
Q

Complement-mediated HSN rxns?

A

II, III

138
Q

Which hypersensitivity reactions involve neutrophils?

A

II, III

139
Q

Which hypersensitivity reaction involves T cells?

A

IV

140
Q

Which hypersensitivity reactions involve macrophages?

A

II, IV

Step 1 Buzzwords (3 decks)

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