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General Pediatrics > Immunology > Flashcards

Immunology Flashcards

1
Q

What are secondary causes to consider when thinking of PID

A

Meds:

  • steroids
  • chemotherapy
  • “natural”

Infections

  • HIV
  • TB
  • Herpes group viruses
  • Measles

Structural

  • anatomic
  • impaired membrane integrity
  • immotile cilia
  • CF
  • indwelling cath

Malignancy:

  • Hodgkin’s
  • leukemia
  • solid tumor
  • myeloma

Other:

  • diabetes
  • renal insufficiency/dialysis
  • hepatic insufficiency
  • malnutrition
  • GERD with aspiration
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2
Q

What is the most common category of PID?

A

Most common:

Humoral deficiencies: 65%

Combined deficiencies (cellular and humoral): 15%
Phagocytic deficiencies: 10%
Cellular deficiencies: 5%
Complement deficiencies: 5%
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3
Q

What are the features of T cell Immune defects

A

Age: 2 - 6 mon

Bacteria: Gram + and -, mycobacteria
Virus: CMV, EBV, Paraflu
Fugi: Candida, PJP

Affected organs: Sinopulmonary, FTT, diarrhea

Associated features: Omenn syndrome, disease post -BCG or VZV vaccine

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4
Q

What are the features of B cell immune defects

A

Age: > 6 mon (after maternal Ig gone)

Bacteria: Encapsulated (strep, Hemophilus)
Viruses: Enterovirus in XLA
Fungi: Giardia, Cryptosporidium

Affected organs: sinopulmonary, GI, arthritis, meningoencephalitis

Associated features: Autoimmune, lymphoma

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5
Q

What are the features of phagocytic immune defects

A

Age: early onset

Bacteria: Staph, Pseud, Serratia
FungI: Candida, Aspergillus

Affected organs: Abscesses, mouth ulcers, osteo

Associated features: delayed cord separation, poor wound healing

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6
Q

What are the features of complement immune defects

A

Age: any age
Bacteria: Pneumococcal, Meningococcal

Affected Organs: Septicemia, meningitis

Associated features: autoimmune

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7
Q

What are the 10 warning signs of Primary Immunodeficiency

A
  1. 4 or more new ear infections w/n 1 yr
  2. 2 or more serious sinus infections w/n 1 year
  3. 2 or more months on abx with little effect
  4. 2 or more pneumonias w/n 1 yr
  5. Failure of an infant to gain weight or grow normally
  6. Recurrent deep skin or organ abscesses
  7. Persistent thrush in mouth or fungal infections on skin
  8. Need for IV abx to clear infections
  9. 2 or more deep seated infections including septicemia
  10. A family hx of PI
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8
Q

Clues on History for a PID

A

Infection:

  • number, frequency, age, severity
  • unusual pathogens?
  • treatment required? IV? hosp?

Autoimmunity:

  • cytopenias
  • colitis
  • arthritis
Malignancy
FTT
Eczema - especially if refractory to txt
Delayed separation of the umbilical cord
Reactions to live viral vaccines
Positive FHx: cosang, infantile deaths, affected maternal uncles
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9
Q

Clues on Physical for PID

A 
FTT
Dysmorphic
Absent tonsils/CLN
Scared TM
Oral Thrush
Lung disease (pneumonia, bronchiectasis)
Splenomegaly
Skin: fungal skin/nail infections, eczema, chronic warts/molluscum, abnormal nails
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10
Q

What do you expect on CBC for pts with PID

A

Lymphs

  • increased in some malignant conditions
  • reduced in many primary/secondary immune def.

Neuts

  • increased in some phagocytic diseases
  • Reduced in autoimmunity and BM failure

Eosinophils
-increased in atopic diseases

Platelets

  • increased in inflammatory conditions
  • reduced in autoimmunity and WAS
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11
Q

How do you do a Humoral Assessment

A

Look at number:

  • Quantitative Ig levels (IgG, IgA, IgM, IgE)
  • Lymphocyte subsets: evaluate B cell numbers (CD19+ cells)

Function:

  • specific abs to antigen which the patient was exposed through vaccination or disease (diptheria, tetanus, measles, mumps, rubella, varicella, EBV, CMV, pnumococcal)
  • Isohemagglutinin (IgM) to Blood group A or B
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12
Q

How do you do a Cellular Assessment

A

Number

  • lymphocyte subsets
  • evaluate CD4 and CD8 cells

Function:

  • lymphocyte proliferation in response to mitogens and antigens (mitogen stimulation assay testing)
  • Adenosine deaminase and purine nucleoside phosphorylase level
  • T-cell receptor excision circles (TRECs)
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13
Q

How do you do a Phagocytic assessment

A

Number:
Neutrophil Counts

Function:

  • Nitroblue tetrazolium test (NBT) or neutrophil oxidative burst index (NOBI)
  • measurement of adhesion markers: CD11 and CD 18 (specialized testing)
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14
Q

How do you do a Complement Assessment

A

Number:

  • C1 esterase inhibitor levels
  • Specific complement levels

Function:

  • Total hemolytic complement (CH50)
  • alternative pathway (AH50)
  • C1 esterase inhibitor function
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15
Q

List 3 types of Humoral (Antibody) Immune Deficiencies

A
  • X-linked agammaglobulinemia (XLA)
  • Common variable immune deficiency (CVID)
  • Transient hypogammaglobulinemia of infancy
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16
Q

What is X-linked Agammaglobulinemia

A
  • Absent B lymphocytes in peripheral blood
  • No B cells = No lymphatic tissue!! (no LN or tonsils on PE)
  • No immunoglobulin produced
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17
Q

What is the gender, age range of pts with XLA and what kind of infections do they get?

A

Gender - Male
Age: 6 - 24 months (when protective maternal IgG disappears
Infections: Sinopulmonary, OM, GI, arthritis, meningitis, sepsis. Encapsulated bacteria (S. pneumo, H. influ) and enteroviral meningoencephalitis

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18
Q

What lab features are seen in XLA

A
  • absent B cells <2 % of lymphocytes
  • absent IgG, IgA, IgM
  • absent Abs to vaccines
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19
Q

How do you manage XLA

A
  • Abx txt of infections
  • IgG repacelment for life (monthly IV or wkly SC). Monitor trough IgG levels aim for lower limit
  • follow PFT and CT chest - at risk for bronchiectasis
  • genetic confirmation and gentic counselling
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20
Q

What is Common Variable Immunodeficiency. What are the clinical features

A
  • most common humoral PID
  • heterogenous disorder (variable)
  • adults but also peak in first years of life

-presence of lymphatic tissue

Clinical Features:

  1. Autoimmunity:
    - cytopenia, IBD/pangastritis, small bowel nodular lymphoidhyperplasia, arthritis, granulomas (lung, liver spleen skin), thyroiditis
  2. Malignancy
    - increased incidence of lymphoreticular and gastric malignancies
  3. Recurrent infections
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21
Q

What kind of infections occur in CVID

A

recurrent bacterial and sinopulmonary infections:
-H. flu, S. pneumo = bronchietctasis and fibrosis in lungs

mycoplasma
enterovirus
giardiasis

22
Q

How do you manage CVID

A
  • similar to XLA
  • Abx txt
  • Immunoglobulin replacement for life
  • Monitor for autoimmunity and malignancy
23
Q

What are Combined Immune Deficiencies and list 6

A

Abnormalities in T and B cell development and function
(in some the humoral deficiency can be related to inability of T cells to instruct B cells)

  • severe combined immunodeficiency (SCID)
  • Wiskott-Aldrich Syndrome (WAS)
  • Ataxia Telangiectasia (AT)
  • Hyper-IgM syndrome
  • X-linked Lymphoproliferative Disease
  • Cartilage Hair Hypoplasia
24
Q

What are the features of SCID

A
  • present by 2 - 6 months of age (die by 1 yr unless txted)
  • Medical emergency
  • Persistent, recurrent, severe opportunistic infections: (bacterial, fungal and viral, sinopulmonary, oral thrush, chronic diarrhea)
  • all lead to FTT
  • absent LN and tonsils
25
Q

What are the lab features of SCID

A

Number:

  • lymphopenia
  • severely reduced T cell numbers
  • B and NK cell numbers can be low, normal or elevated

Function:
-low or absent T cell function
(lymphocyte proliferation in response to mitogens and antigens)
-absent Abs to vaccines

26
Q

What is the management of SCID (6)

A

Treat and prevent infections:

  • aggressive antimicrobial therapy
  • Immunoglobulin replacement therapy
  • PJP prophylaxis
  • CMV negative irradiated blood products
  • Strict protective isolation

Most important
-new immune system = BMT

27
Q

Features of Wiskott-Aldrich Syndrome

A

X-linked condition with triad of:

  • Thrombocytopenia
  • Eczema
  • Recurrent pyogenic infections in infancy
  • Watery/blood diarrhea & petechiae in first months
  • bacterial infection with encapsulated organism
28
Q

What are the lab features of WAS

A

Number:

  • Thrombocytopenia
  • Low IgM, variable IgG, high IgA, high IgE

Function:

  • poor ab response to vaccines
  • decreased T cell function
29
Q

Management of WAS

A
  • Immunoglobulin replacement therapy
  • Abx proph
  • BMT
30
Q

What are the features of Ataxia Telangiectasia (5)

A
  1. Autosomal Recessive
  2. Ataxia (cerebellar)
    - starts around 18 months of age
    - wheelchair by teenage years
  3. Telangictasia
    - prominent on face, conjunctiva, ear lobs
    - appears at 2 - 4 yrs of age
  4. Progressive neurodegeneration
  5. Immune deficiency
    - not as clinically significant
    - Can have recurrent sinopulmonary infections and bronchiectasis
31
Q

Lab features of Ataxia Telangiectasia?

A

Number:

  • increased AFP
  • decreased T cell numbers
  • Absent IgA in 80%

Function:

  • decreased T cell function
  • Can have poor Ab response to some vaccines
32
Q

What investigation must you avoid with pts with AT

A
  • avoid X-ray and CT

- cells have increased sensitivity to irradiation

33
Q

How do you manage AT

A
  • supportive, BMT not possible
  • Immunoglobulin replacement therapy in those with demonstrated humoral deficiencies
  • watch for malignancy: 15% may develop malignancy especially lymphoma
34
Q

What are the features of DiGeorge Syndrome

A

-cellular deficiency

CATCH 22:
- cardiac defects, abnormal facial features, thymic hypoplasia, cleft palette and midline abnormalities, hypocalcemia, 22q.11 deletion (3rd & 4th pharyngeal pouch)

  • FTT
  • developmental delay
  • Psychiatric issues (Schizophrenia)
35
Q

What are the facial features of DiGeorge

A
  • hooded eyelids
  • bulbous nasal tip
  • hypertelorism
  • low set prominent posteriorly rotated ears
  • notched pinnae
  • reduced helix formation
  • migrognathia
  • short philtrum
  • bifid uvula
  • high arched palate
36
Q

What are the Lab features of DiGeorge

A

Number:
-mildly reduced CD4+ and CD8+ T cells (improves with age)

Function:

  • normal or mildly reduced T cell function
  • may have poor Ab response to vaccines (uncommon)
37
Q

Management of immune defects in DiGeorge

A
  • most only have mild T cell deficiency which improves with age
  • some have severe T cell deficiency (treat like SCID)
  • blood products should be CMV neg and irradiated
  • Should not receive live viral vaccines until immune competence demonstrated
38
Q

List 3 Phagocytic Immune Deficiencies

A

Chronic Granulomatous Disease
Hyper IgE Syndrome (Job’s)
Leukocyte Adhesion Defects (LAD)

39
Q

List 2 innate Immune Deficiencies

A

IRAK4 Deficiency

Mendelian susceptibility to myocbateria

40
Q

What are the features of Chronic Granulomatous Disease

A

Defect in NADPH oxidase
-required for effective phagocytic killing of certain pathogens

65% X-linked
-remainder are AR

  • recurrent bacterial and fungal infections since infancy
  • Abscesses and granulomas (skin, LN, lung, liver, GI, GU with bladder obstruction)
  • inflammation and colitis
41
Q

What organisms are pts with CGD susceptible to? (6)

A

Catalase positive pathogens

  • S. aureus
  • Aspergillus
  • Nocardia
  • Serratia marcescens
  • Burkholderia cepacia
  • Salmonella
42
Q

What are the lab features of CGD

A

Number:
normal or increased neutrophils

Function:
-Abnormal NOBI or NBT

43
Q

What is the Management of CGD

A
  • Aggressive abx for current infections
  • Antibacterial and antifungal proph
  • Anti-inflammatory txt (for colitis)
  • BMT
  • Genetic Counselling
44
Q

What are the features of Hyper IgE Syndrome (Job’s) (6)

A
  1. AD mutation in STAT3 OR AR in Dock8
  2. Recurrent abscesses in skin, joint, lungs
    - ‘cold boils’ (no pain, heat or erythema)
    - S. aureus
  3. Eczema
  4. Coarse facial features
  5. Delayed shedding of teeth
  6. Bone fractures, scoliosis, joint hyperlaxity
45
Q

What is the management of Hyper IgE syndrome

A
  • treat infections

- anti-staphylococcal prophylaxis (Cotrimoxazole)

46
Q

What are the lab features of Hyper IgE Syndrome

A

Number:

  • Very high IgE (> 20,000) (not required to be high to have hyper IgE)
  • Eosinophilia

Function:
-can have poor antibody response to vaccines

47
Q

What is the features of Leukocyte Adhesion Defects

A

-very rare
-deficiency in adhesion molecules:
Abnormal neutrophil migration and penetration into tissue
Neutrophils remain in the blood, unable to migrate to site of infection
-delayed separation of umbilical cord > 4 wks
-staphlococcal infections: dental gingivitis, intestinal

48
Q

Laboratory Features of LAD

A

Number:
-Neutrophilia: Can be > 100,000 even when no infection

Function:
-absent surface adhesion molecules CD11 and CD18

49
Q

What is the management for LAD

A
  • Antibiotics

- Bone Marrow transplant

50
Q

What are the defects in early (C1, C2 and C4) components

What are the defects in late (C5 - 9) components

A

C1, C2 , C4:
Rheumatic disease, lupus, infections rare

C5 - C9
invasive neisserial and pneumococcal infections

51
Q

What is the management for complement deficiencies

A
  • Abx prophylaxis

- Immunizations (meningococcal & pneumococcal)

52
Q

What are the lab features of complement deficiencies

A

Number:

  • normal C3, C4 (don’t send)
  • measuring other specific complement levels only done in specialized labs

Function:

  • Abnormal CH50 (total hemolytic complement)
  • check if alternative pathway (AH50) if CH50 normal and still suspect complement deficiency

General Pediatrics (19 decks)

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