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Random Notes for Royal College Exam > Allergy and Immunology > Flashcards

Allergy and Immunology Flashcards

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Royal College: Emergency Medicine flashcards
1
Q

What is penicillin anaphylaxis mediated by?

A

IgE against a major determinant

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2
Q

What is the most cost-effective test of T cell function?

A

Candida skin test

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3
Q

Which immunodeficiency may lead to anaphylaxis in a patient given IVIG?

A

Selective IgA deficiency

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4
Q

What are early complement deficiencies (C1, C2, C4) associated with?

A

Rheumatic diseases, lupus, infections rare

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5
Q

What are late complement deficiencies (C5-9) associated with?

A

Invasive neisserial (meningitis and gonorrhea) and pneumococcal infections

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6
Q

How long should live vaccines be deferred post IVIG therapy?

A

11m

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7
Q

What defects are present in X-linked agammaglobulinemia?

A
  1. Absent B lymphocytes
  2. No Ig production
  3. BTK mutation
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8
Q

What are the clinical characteristics for X-linked agammaglobulinemia?

A
  1. Males @ 6-24mo
  2. Recurrent sinopulmonary, OM, GI, arthritis, meningitis, sepsis
  3. Encapsulated bacteria: strep pneumo, Hib
  4. Enteroviral meningoencephalitis
  5. Absent lymph nodes and tonsils
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9
Q

What is the inheritance pattern for X-linked agammaglobulinemia?

A

X linked recessive

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10
Q

What are the clinical features of common variable immunodeficiency?

A
  1. Recurrent bacterial and sinopulmonary infections
  2. Can lead to bronchiectasis and lung fibrosis
  3. Organisms: H flu, strep pneumo, mycoplasma, enteroviruses, giardiasis
  4. Autoimmunity (20-25%)
    a) cytopenias
    b) IBD-like, gastritis, or small bowel nodular lymphoid hyperplasia
    c) arthritis
    d) granulomas: lung, liver, spleen, skin
    e) thyroiditis
  5. Malignancy esp. lymphoreticular and gastric
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11
Q

What are the laboratory features of CVID?

A
  1. Decreased serum IgG
  2. Normal or reduced IgA and IgM
  3. Normal or low B-cells
  4. Decreased/absent antibodies to vaccines
  5. Low isohemagglutinins
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12
Q

What is the most common inheritance pattern for severe combined immunodeficiency (SCID)?

A

X-linked

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13
Q

What are the clinical features of SCID?

A 
1. Presents @ 2-6mo, fatal by 1yo if untreated
2, Persistent, recurrent, severe opportunistic infections:
a) bacterial, fungal, and viral
b) sinopulmonary
c) oral thrush
d) chronic diarrhea
3. FTT
4. Absent lymph nodes & tonsils
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14
Q

What are the laboratory features of SCID?

A
  1. Lymphopenia
  2. Severely reduced T cell numbers
  3. Low, normal, or elevated B and NK cell numbers
  4. Low or absent T cell function
  5. Absent antibodies to vaccine
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15
Q

What is the management of SCID?

A
  1. Immunoglobulin replacement therapy
  2. Antibiotic prophylaxis
  3. Bone marrow transplant
  4. Protective isolation
  5. Aggressive antibiotic therapy for infections
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16
Q

What is the inheritance pattern of Wiskott-Aldrich syndrome?

A

X-linked

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17
Q

What are the clinical features of Wiskott-Aldrich syndrome?

A
  1. Thrombocytopenia
  2. Eczema
  3. Recurrent pyogenic infections in infancy
  4. Watery/bloody diarrhea and petechiae in 1st months
  5. Bacterial infections w/ encapsulated organisms
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18
Q

What are the laboratory features of WAS?

A
  1. Thrombocytopenia
  2. Low IgM
  3. Variable IgG
  4. high IgA and IgE
  5. Poor antibody responses to some vaccines
  6. Decreased T cell function
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19
Q

What is the management for WAS?

A
  1. Immunoglobulin replacement therapy
  2. Antibiotic prophylaxis
  3. Bone marrow transplant
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20
Q

What is the inheritance pattern of ataxia telangiectasia?

A

AR

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21
Q

What are the clinical features of AT?

A
  1. Ataxia (starts 18mo, wheelchair bound by teen)
  2. Telangiectasia (face, conjunctiva, ear lobes by 2-4yo)
  3. Progressive neurodegeneration
  4. Recurrent sinopulmonary infections & bronchiectasis
  5. 15% malignancy esp. lymphoma
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22
Q

What are the laboratory features of AT?

A
  1. Increased AFP
  2. Decreased T cell number and function
  3. Absent IgA 80%
  4. Poor antibody responses to some vaccines
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23
Q

What is the management of AT?

A
  1. Supportive

2. Ig replacement therapy is humoral deficiency

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24
Q

What are the clinical features of DiGeorge?

A
  1. Cardiac defects esp. interrupted aortic arch
  2. Abnormal facial features
  3. Thymic hypoplasia
  4. Cleft palate and midline abnormalities
  5. Hypocalcemia
  6. 22q11 deletion
  7. FTT
  8. Developmental delay
  9. Psych issues i.e. Schizophrenia
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25
Q

What are the laboratory features of DiGeorge?

A
  1. Mildly reduced CD4+ and CD8+ T cell numbers
  2. Normal or mildly reduced T cell function
  3. May have poor antibody responses to vaccines (uncommon)
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26
Q

What is the management of immune defects in DiGeorge?

A
  1. Treat like SCID if complete DiGeorge
  2. CMV-negative, irradiated blood products only
  3. No live vaccines until confirmed immune competent
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27
Q

What is the inheritance pattern of CGD?

A

X-linked (65%)

rest AR

28
Q

What is the defect in CGD?

A

NADPH oxidase

29
Q

What pathogens are patients with CGD susceptible to?

A

Catalase positive pathogens

  1. S aureus
  2. Aspergillus
  3. Nocardia
  4. Serratia marcescens
  5. Burkholdria cepacia
  6. Salmonella
30
Q

What are the clinical features of CGD?

A
  1. Abscesses and granulomas (skin, lymph nodes, lung, liver, GI, GU)
  2. Inflammation and colitis
31
Q

What are the laboratory features of CGD?

A
  1. Abnormal NOBI (neutrophil oxidative burst index)
  2. Abnormal NBT (nitro-blue tetrazolium text)
  3. Normal or increased neutrophil numbers
32
Q

What is the management of CGD?

A
  1. Aggressive antibiotic therapy for current infections
  2. Antibacterial and antifungal prophylaxis
  3. Anti-inflammatory treatment
  4. BMT
  5. Genetic counselling
33
Q

What is the inheritance pattern of hyper IgE syndrome (Job’s)?

A

AD (STAT3)

AR (Dock8)

34
Q

What are the clinical features of hyper IgE syndrome (Job’s)?

A
  1. Recurrent abscesses in skin, joints, lungs
    • “Cold boils” (no pain, heat, or redness)
    • S. aureus
  2. Eczema
  3. Coarse facial features
  4. Delayed shedding of teeth
  5. Bone fractures, scoliosis, joint hyperlaxity
35
Q

What is the management of Job’s?

A
  1. Treat infections

2. Anti-staphylococcal prophylaxis (septra)

36
Q

What are the laboratory features of hyperIgE syndrome?

A
  1. IgE > 20 000
  2. Eosinophilia
  3. Poor antibody responses to vaccines possible
37
Q

What are the clinical features of leukocyte adhesion defects (LAD)?

A
  1. Delayed umbilical cord separation

2. Staphylococcal infections (Dental, gingival, intestinal)

38
Q

What is the defect in LAD?

A

Deficiency in adhesion molecules with abnormal neutrophil migration and penetration into tissue

39
Q

What are the laboratory features of LAD?

A
  1. Neutrophilia (>100 000 even when no infection)

2. Absent surface adhesion molecules CD11 and CD18

40
Q

What is the management of LAD?

A
  1. Antibiotics

2. BMT

41
Q

What is the inheritance pattern of most complement deficiencies?

A

AR

42
Q

What is the management for complement deficiencies?

A
  1. Antibiotic prophylaxis

2. Immunizations (meningococcal & pneumococcal)

43
Q

What are laboratory features of complement deficiencies?

A
  1. Normal C3, C4 levels
  2. Abnormal CH50
  3. Check AH50 if CH50 normal and suspect complement deficiency
44
Q

What clinical features are associated with chronic mucocutaneous candidiasis?

A
  1. Persistent candida in skin, nails, mucous membranes

2. Endocrine autoimmunity (parathyroid, adrenal > thyroid, pancreas, gonads)

45
Q

What syndrome is asosciated with chronic mucocutaneous candidiasis?

A

Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED)
APS-1 (autoimmune polyendocrine syndrome tpe 1)

46
Q

What is the inheritance pattern of hyper IgM syndrome?

A

X-linked

47
Q

What is the clinical presentation of hyper IgM syndrome?

A
  1. Bacterial infections in 1st year
  2. Autoimmunity, and PJP pneumonia
  3. Malignancy i.e. lymphoma later in life
48
Q

What are the laboratory features of hyper IgM syndrome?

A
  1. Neutropenia
  2. High IgM and low IgG (reverse ratio)
  3. B cell defect - do not produce antibodies
  4. Normal number of T and B cells
49
Q

What is the management of hyper IgM syndrome?

A
  1. Immunoglobulin replacement therapy
  2. PJP antibiotic prophylaxis
  3. BMT
50
Q

What is the inheritance of X-linked lymphoproliferative disease?

A

X-linked

51
Q

What are the clinical features of X-linked lymphoproliferative disease?

A
  1. Selective inability to respond to EBV infection
  2. 75% develop severe/fatal infectious mononucleosis w/ hepatic necrosis and bone marrow failure
  3. Increased frequency of lymphoma
52
Q

What is the treatment of X-linked lymphoproliferative disease?

A
  1. Ig replacement therapy

2. BMT

53
Q

What clinical features are associated with cartilage hair hypoplasia?

A
  1. Short limbed dwarfism
  2. Short pudgy hands
  3. Hyper-extensible joints
  4. Sparse light hair
  5. Hirschsprung’s disease
  6. Immune defect incld. SCID
  7. Increased freq. of malignancy
54
Q

What is the inheritance pattern of IRAK4 deficiency?

A

AR

55
Q

What are the clinical features of IRAK4 deficiency?

A
  1. Recurrent, severe infections (pneumonia, meningitis, septicemia) due to S. pneumonia, S. aureus in infancy
  2. No major infections in adulthood
56
Q

What are the laboratory features of IRAK4 deficiency?

A
  1. Normal T&B lymphocytes
  2. Normal phagocyte function
  3. Normal complement function
57
Q

What is the inheritance pattern of hereditary angioedema?

A

AD

58
Q

What are the clinical features of hereditary angioedema?

A
  1. Recurrent episodes of localized swelling of face, extremities, or internal organs (GI tract)
  2. No pain, heat, or itch
59
Q

What is the laboratory feature of hereditary angioedema?

A

Decreased C1 esterase inhibitor level

60
Q

What is the management of hereditary angioedema?

A
  1. Concentrates of C1 inhibitor infusions during attacks

2. Androgens could be considered for prophylaxis

61
Q

What is the inheritance pattern for ALPS (autoimmune lymphoproliferative syndrome)?

A

AD

62
Q

What are the clinical features of ALPS?

A
  1. Autoimmune cytopenias
  2. Lymphoproliferation (spleen, lymph nodes)
  3. Increased incidence of malignancy
63
Q

What are the laboratory features of ALPS?

A
  1. Anemia, neutropenia and/or thrombocytopenia
  2. Elevated IgG levels
  3. Elevated % T cells not expressing CD4 or CD8 (“double negative”)
  4. Normal antibody responses to vaccines
  5. Often normal T cell function
64
Q

What is the management of ALPS?

A

immune suppression

65
Q

What are the clinical features of IPEX?

A 
I = immune dysregulation
P = polyendocrinopathy (neonatal or infantile insulin dependent DM)
E = enteropathy (severe colitis)
X = X linked
66
Q

What is the treatment of IPEX?

A
  1. immune suppression

2. BMT

67
Q

What should be avoided in X-linked agammaglobulinemia?

A

Live vaccines as can get paralysis secondary to live polio vaccine

Random Notes for Royal College Exam (23 decks)

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