Acaai Review For The Allergy & Immunology Boards Flash Cards Chapter 6

Front 1

C1q

Back 1

Which complement component is decreased in acquired, but not hereditary, angioedema?

Front 2

Ecallantide (Kalbitor)

Back 2

Which medication approved for treatment of acute attacks of HAE carries a black box warning for anaphylaxis?

Front 3

Selective IgA deficiency

Back 3

What is the most common primary immunodeficiency disorder?

Front 4

T-B-NK+

Back 4

What is the lymphocyte phenotype (T/B/NK) with SCID secondary to a defect secondary to Artemis?

Front 5

AIRE gene resulting in autoimmune type symptoms, including endocrinopathies, hypoparathyroidism, hypoadrenalism

Back 5

What gene defect can lead to chronic mucocutaneous candidiasis? Other associated clinical features?

Front 6

BTK; there is absent or limited lymphoid tissue secondary to no germinal centers

Back 6

What is the mutation that causes X-linked agammaglobulinemia? Clinically describe the lymphoid tissue present?

Front 7

CXCR4, which is needed in bone marrow trafficking of progenitor cells

Back 7

What is the molecular defect in WHIM syndrome?

Front 8

TMP-SMX and penicillin V

Back 8

What are two prophylactic antibiotic options for IRAK4/MyD88 deficiency?

Front 9

X-linked

Back 9

What is the pattern of inheritance for properidin deficiency?

Front 10

Herpes viruses

Back 10

With NK cell deficiency, what organism(s) causes recurrent infections?

Front 11

Thrombocytopenia, eczema, and recurrent infections; patients have a risk of developing EBV-related lymphoma

Back 11

What is the triad involved with Wiskott-Aldrich syndrome? Patients with this syndrome have an increased risk of developing what condition?

Front 12

HPV and nontuberculosis mycobacterial infection, myelodysplasia, pulmonary alveolar proteinosis, and congenital lymphedema.

Back 12

Patients with GATA2 deficiency have an increased risk of developing what?

Front 13

By 2 to 4 years old

Back 13

Transient hypogammaglobulinemia of infancy usually resolves by what age?

Front 14

LAD 2

Back 14

Which defect in adhesion and chemotaxis causes developmental delay, microcephaly, and short stature?

Front 15

ADA (T-, B-, and NK-)

Back 15

Which form of SCID has the worst prognosis after stem cell transplant?

Front 16

CD45RO+ T lymphocyte and neutrophil engraftment

Back 16

What cells are involved in acute GVH reaction?

Front 17

CCR5 and CXCR4

Back 17

Name the two HIV coreceptors.

Front 18

CCR5Δ32 mutation

Back 18

Which mutation has been associated with HIV resistance (double allelic) and slower disease progression (single allelic)?

Front 19

HLA-B*5701

Back 19

Which HLA allele is associated with an increased risk of abacavir hypersensitivity?

Front 20

WHIM syndrome

Back 20

An autosomal dominant mutation in the gene for HIV coreceptor CXCR4 has been linked to what congenital immunodeficiency?

Front 21

Eotaxin-3

Back 21

Which chemokine has been strongly implicated in the development of eosinophilic esophagitis?

Front 22

SLP

Back 22

SNP variants in what cytokine are overexpressed in EoE biopsy tissue?

Front 23

Esophagus

Back 23

Which segment of the GI tract is devoid of eosinophils under normal conditions?

Front 24

The most common mutation in c-kit is at codon 816 with a substitution of valine for aspartic acid (Asp816Val or D816V)

Back 24

What is most common activating point mutation in c-kit associated with systemic mastocytosis?

Front 25

Aggressive systemic mastocytosis

Back 25

Which type of systemic mastocytosis is associated with cytopenias and liver injury?

Front 26

Bone marrow biopsy showing multifocal, dense infiltrates of mast cells (>15 mast cells in aggregates)

Back 26

What is the major criterion for systemic mastocytosis (SM)?

Front 27

Babesia microti and/or Anaplasma phagocytophilum (causing human granulocytic anaplasmosis

Back 27

Which pathogenic organisms share the same vector (Ixodes tick) and can cause coinfection in patients with Lyme disease?

Front 28

Microscopy: thin smear

Back 28

How is the definitive diagnosis of babesiosis made?

Front 29

1-2 weeks for IgM and 4-6 weeks for IgG

Back 29

When would you expect anti-IgM and anti-IgG to appear after B. burgdorferi infection?

Front 30

Anti-TNFα antibody and TNFα receptor antibody

Back 30

What monoclonal therapy agents can cause reactivation of tuberculosis?

Front 31

TLR2 and TLR4; MyD88

Back 31

What TLR receptors are important in the innate immune response to tuberculosis? What adapter protein do they activate?

Front 32

IL-12, IL-18, and IL-23

Back 32

What cytokines do tuberculosis-infected macrophages secrete to stimulate naïve T cells to differentiate into T-helper 1 cells?

Front 33

STAT1

Back 33

Activation of which transcription factor is needed in order to transcribe IFN-γ regulated genes?

Front 34

Number of skin lesions and smears of skin lesions

Back 34

On what criteria does the WHO base its classification of leprosy?

Front 35

They have a selective inability to mount a cellular response to M. leprae

Back 35

If a person has numerous (>6) poorly demarcated lesions, what does it say about their immune response to Mycobacterium leprae?

Front 36

Multibacillary leprosy (MB)

Back 36

What WHO classification is most likely to develop a type II reaction (erythema nodosum leprosum)?

Front 37

HAV is not hepatoxic; HAV-specific CD8 + cells and NK cells cause injury to hepatocytes

Back 37

How does hepatitis A virus injure hepatocytes?

Front 38

When hepatitis B is in the immune active stage (abnormal ALT and HBV DNA >20,000 IU/mL)

Back 38

When should chronic hepatitis B be treated in children?

Front 39

Type II, mixed polyclonal and monoclonal globulins (mixture of monoclonal IgM and polyclonal IgG)

Back 39

What type of cryoglobulinemia is associated with hepatitis C?

Front 40

Congenital syphilis, syphilis in pregnant women, and neurosyphilis

Back 40

What are the indications to undergo penicillin desensitization in penicillin-allergic patients who require antibiotic treatment for syphilis?

Front 41

Early syphilis: tetracyclines, macrolides, or ceftriaxone; latent syphilis: doxycyline

Back 41

What are the options for treatment of syphilis in penicillin-allergic patients?

Front 42

Acute febrile reaction associated with headache and myalgias within the first 24 hours of penicillin treatment for syphilis.

Back 42

What is a Jarisch-Herxheimer reaction?

Front 43

Serial evaluation should occur every 3-6 months until the effusions resolves, hearing loss is documented, or structural changes of the TM or middle ear are noted

Back 43

How often should otitis media with effusion be evaluated?

Front 44

3 months

Back 44

When should you obtain a baseline hearing test after the onset of otitis media with effusion?

Front 45

Haemophilus influenzae

Back 45

In acute otitis media with conjunctivitis, what pathogen is most likely the culprit?

Front 46

>3 episodes within 6 months or >4 episodes within 1 year with at least 1 episode in the previous 6 months

Back 46

After how many episodes of acute otitis media should a referral be placed to ENT for possible tympanostomy placement?

Front 47

Toxic-appearing, moderate-severe otalgia, and temperature >102.2°F

Back 47

What severe signs and symptoms of acute otitis media in a patient ≥2 years old would prompt antibiotic therapy instead of observation?

Front 48

Aspergillus fumigatus

Back 48

What is the most common cause of infectious fungal sinusitis?

Front 49

Sinus opacification due to accumulation of allergic mucin that, under microscopy, are filled with eosinophils, Charcot-Laden crystals, and fungal hyphae

Back 49

What is the hallmark of allergic fungal sinusitis?

Front 50

(1) Persistent signs/symptoms of ARS for >10 days without evidence of improvement; (2) Fever >102°F plus purulent rhinorrhea or facial pain that has lasted for at least 3-4 consecutive days; (3) URI symptoms for 5-6 days followed by worsening sign/symptoms, that is, "double sickening"

Back 50

Which three clinical presentations best identify patients with acute bacterial versus viral rhinosinusitis?

Front 51

Amoxicillin-clavulanate; high-dose amoxicillin-clavulanate is indicated if the patient lives in an area with high endemic rates of resistant S. pneumoniae, has a fever >102°F, faces a threat of suppurative complications, participates in day care, is younger than 2 or older than 65 years old, was recently hospitalized, was treated with antibiotics within the previous month, or is immunocompromised.

Back 51

What is the treatment of choice for acute bacterial rhinosinusitis? When are higher doses indicated?

Front 52

≥2 episodes in 1 year or ≥3 episodes ever, with radiographic clearing of densities between episodes

Back 52

What is the definition of recurrent pneumonia in children?

Front 53

Pneumonia that occurs ≥48-72 hours after endotracheal intubation

Back 53

What is the definition of ventilator associated pneumonia?

Front 54

More than one case of pneumonia per decade

Back 54

What is the definition of recurrent pneumonia in adults?

Front 55

Rales

Back 55

The presence of what physical exam finding helps to distinguish bronchiolitis from viral-induced wheezing?

Front 56

Supportive care; bronchodilators, corticosteroids, and leukotriene receptor antagonists have not been shown to be consistently effective

Back 56

What is the typical management of bronchiolitis?

Front 57

Parainfluenza virus type 1

Back 57

Which type of parainfluenza virus most often causes acute laryngotracheitis?

Front 58

Anti-histone antibodies

Back 58

Which antibodies are associated with drug-induced lupus?

Front 59

Anti-Ro/SSA and/or anti-La/SSB

Back 59

What antibodies are associated with neonatal lupus?

Front 60

Arthritis, myositis, obstructive lung disease, and "mechanic's hands" (cracked, furrowed hands)

Back 60

What symptoms are associated with the anti-synthetase syndrome of dermatomyositis or polymyositis?

Front 61

"Full house" immunofluorescence with IgG, IgA, IgM, C3, and C1q

Back 61

What is the typical finding on biopsy of lupus nephritis?

Front 62

Anticentomere antibody

Back 62

What autoantibody is associated with CREST syndrome?

Front 63

Pauciarticular JIA

Back 63

Which type of JIA has the highest risk for uveitis?

Front 64

RA, SLE, polymyositis, and scleroderma

Back 64

What other connective tissue disorders are associated with secondary Sjogren's syndrome?

Front 65

Triad of RA, neutropenia, and splenomegaly

Back 65

What is Felty syndrome?

Front 66

Ileocecal B-cell lymphoma

Back 66

The incidence of which lymphoma is very high in patients with X-linked lymphoproliferative syndrome (XLP)?

Front 67

Hepatitis C virus, with types II and III

Back 67

Which viral infection is associated with cryoglobulinemia, and with what types?

Front 68

Reed-Sternberg cell (B-lymphocyte origin)

Back 68

What type of cell is typically seen in Hodgkin's lymphoma?

Front 69

c-myc gene translocation, found at 8q24

Back 69

Which gene translocation is often seen with Burkitt's lymphoma?

Front 70

The most common mutation in c-kit is at codon 816 with a substitution of valine for aspartic acid (Asp816Val or D816V); other mutations have been identified but are uncommon

Back 70

What is most common activating-point mutation in c-kit associated with systemic mastocytosis?

Front 71

C5a

Back 71

During a TRALI, which chemotactic factor is mainly responsible for pulmonary leukosequestration?

Front 72

I antigen

Back 72

Cold-reactive AIHA is seen when IgM binds to which polysaccharide antigen on RBCs?

Front 73

In the 28th week of gestation or within 72 hours after an antepartum event involving placenta/fetal trauma or fetomaternal bleeding.

Back 73

When should anti-D immunoglobulin be administered to pregnant women?

Front 74

CD4 and CD8

Back 74

Which CD markers are alpha/beta double-negative T cells (DNTs) lacking?

Front 75

FAS

Back 75

What is the most common genetic cause of autoimmune lymphoproliferative syndrome (ALPS)?

Front 76

Hyperthyroidism, exophthalmos, and pretibial myxedema

Back 76

What are three main manifestations of Grave's disease?

Front 77

IL-17 and IL-22

Back 77

Candidiasis in autoimmune polyendocrine syndrome-1 is due to antibodies against which target?

Front 78

CD4+, CD25+, FoxP3+, and T-regulatory cells

Back 78

Which subset of T cells is significantly decreased or absent in IPEX?

Front 79

Stiff-Person syndrome

Back 79

Antibodies against GAD are found in which neurologic condition?

Front 80

Membranous nephropathy

Back 80

Subepithelial deposits are found in which type of immune-mediated kidney disease?

Front 81

Supportive therapy only

Back 81

What is the treatment of choice for poststreptococcal glomerulonephritis?

Front 82

Hepatitis C

Back 82

Which infectious disease is most closely associated with essential mixed cryoglobulinemia

Front 83

Pauci-Immune crescentic glomerulonephritis (due to drug-induced ANCA-associated vasculitis)

Back 83

Minocycline use is associated with which type of nephritis?

Front 84

Subepidermal IgA granular deposits within dermal papillae on immunofluorescence

Back 84

What is the characteristic pathology of dermatitis herpetiformis?

Front 85

Crohn's disease

Back 85

ASCA antibodies are more likely to be positive in which type of inflammatory bowel disease?

Front 86

IL-1

Back 86

What interleukin is responsible for the inflammation for many autoinflammatory diseases?

Front 87

Inflammasome

Back 87

What complex of proteins within the innate immune system is a common mediator of autoinflammatory syndromes?

Front 88

TRAPS

Back 88

Which autoinflammatory syndrome has attacks that can last upward of weeks?

Front 89

Stress and vaccinations

Back 89

What are triggers for attacks in patients with hyper-IgD syndrome (HIDS)?

Front 90

Anakinra, rilonacept, and canakinumab

Back 90

What monoclonal antibodies are used to target IL-1?

Front 91

Evaporative cooling test (NOT the ice cube test)

Back 91

What test is needed to elicit the cold urticaria in PLCγ2-associated antibody deficiency and immune dysregulation (PLAID) and familial cold autoinflammatory syndrome (FCAS)?

Front 92

Muckle-Wells syndrome

Back 92

A 46-year-old woman with a history of progressive hearing loss presents with recurrent episodes of fever, diffuse urticaria, and diffuse joint pains triggered by cold temperatures. Laboratory studies reveal proteinuria and kidney biopsy reveals AA deposits. What is the diagnosis?

Front 93

Serum protein electrophoresis (SPEP) to look for a paraprotein peak

Back 93

A 60-year-old woman presents with renal failure and nephrotic syndrome. A renal biopsy demonstrates apple-green birefringence and polarized light, confirming amyloidosis. What is the best test to rule out a possible multiple myeloma?

Front 94

Hepatitis C virus, hepatitis B virus, HIV, herpesviruses, and mycobacteria

Back 94

What infections are associated with type II and type III cryoglobulinemia?

Front 95

Rheumatoid factor (type I lacks RF, while types II and III contain RF)

Back 95

The presence of what antibody distinguished type I from types II and III cryglobulinemia?

Front 96

Sarcoidosis (but you need the occupational history to diagnose berylliosis)

Back 96

Berylliosis is histologically indistinguishable from which other pulmonary granulomatous syndrome?

Front 97

c-ANCA (proteinase 3 or PR3)

Back 97

What type of ANCA antibody is found in 75- 90% of patients with granulomatosis with polyangiitis?

Front 98

GLILD nodules are larger and randomly distributed, whereas sarcoidosis nodules are smaller and perilymphatic.

Back 98

What are the distinguishing characteristics of pulmonary nodules in granulomatous-lymphocytic interstitial lung disease (GLILD) versus sarcoidosis?

Front 99

Tuberculosis, histoplasmosis, blastomycosis, and coccidiomycosis

Back 99

What are the classical infectious causes of granuloma formation?

Front 100

ACE, 1,25-dihydroxy vitamin D, osteoporin, IFNγ, and TNFα

Back 100

What secreted molecules are important in the formation of granulomas?

Front 101

Chronic granulomatous disease and CVID

Back 101

What primary immunodeficiencies are associated with granuloma formation?

Front 102

HLA-C

Back 102

Which additional allele, aside from HLA-A, HLA-B, HLA-DRB1, is assessed for adult donors as opposed to umbilical cord blood?

Front 103

Aspirin-exacerbated respiratory disease (AERD), Churg-Strauss syndrome, and Kartagener syndrome

Back 103

Name three diseases other than cystic fibrosis that are associated with nasal polyps.

Front 104

Homozygous F508del

Back 104

For which mutation is lumacaftor-ivacaftor FDA approved?

Front 105

≥60 Eq/L in at least two measurements

Back 105

What sweat chloride result is diagnostic of CF in children?