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POD Exam 3 > Immunodeficiency - Dr. Carpenter > Flashcards

Immunodeficiency - Dr. Carpenter Flashcards

1
Q

Primary Immunodeficiency: Defects in Lymphocyte Maturation (3 examples)

A

SCID, X-linked agammaglobulinemia, DiGeorge Syndrome

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2
Q

Primary Immunodeficiency: Defects in Lymphocyte Activation and Function (3 examples)

A

X-linked hyper-IgM syndrome, Common variable immunodeficiency, Bare lymphocyte syndrome

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3
Q

Primary Immunodeficiency: Defects in Innate Immunity (4 examples)

A

CGD, Leukocyte Adhesion Deficiency, complement deficiencies (SLE), Chediak-Higashi Syndrome

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4
Q

Primary Immunodeficiency: Lymphocyte abnormalities associated with other diseases (2 examples)

A

Wiskott-Aldrich Syndrome, Ataxia-telangiectawsia

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5
Q

Pyogenic bacterial infections, enteric bacterial and viral infections are common consequences of …

A

B-cell deficiencies (pyogenic bacterial infections and viral infections are common in compliment deficiencies too)

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6
Q

Absent or reduced follicles and germinal centers in lymphoid organs are histopathological and laboratory findings in …

A

B-cell deficiencies

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7
Q

Reduced DTH reactions to common antigens is a histopathological and laboratory finding in …

A

T-cell deficiencies

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8
Q

T-cell deficiencies are seen in what area of lymph tissue?

A

paracortical

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9
Q

Viral-associated malignancies are common consequences in …

A

T-cell deficiencies

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10
Q

Viral and intracellular microbial infections (ex. pneumocystits jiroveci, fungi, atypical mycobacteria) are common consequences seen in …

A

T-cell deficiencies

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11
Q

Cell type most important in pyogenic infections (encapsulated bugs for example)

A

B- Cells

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12
Q

Cell mediated immunity / T- cell immunity is important in malignancy. True or false?

A

True

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13
Q

Name three consequences of all types of immunodeficiencies:

A

1) Increased susceptibility to newly acquired infections
2) Reactivation of latent infections (CMV, EBV, TB)
3) Increased incidences of cancer

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14
Q

Severe (hospital status), persistent, unusual, recurrent infections would make a good doctors suspect…

A

Immunodeficiency (haha i made this notecard just to piss off Jacob)

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15
Q

Congenital primary immunodeficiencies are caused by a genetic defect that leads to block in maturation. Incidence is 1:500. The inheritance pattern can be AR but usually it is…

A

X-linked (ex: SCID)

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16
Q

X- linked SCID functional deficiencies

A

Decreased: T-cells and serum IG

B cell: wnl (aka be cells are normal aka gucci AF)

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17
Q

Most common form of SCID accounting for 50-60% of all cases?

A

X- linked SCID

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18
Q

X- linked SCID mechanism of defect

A

Mutation/ missing common gamma chain receptor for cytokine: decreased T-cell maturation; No IL-7

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19
Q

IL-7 is responsible for

A

development and maturation of pro-T-cells

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20
Q

IL-15 is responsible for

A

Development of NK cells

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21
Q

Low levels of IL-15 due to gamma chain mutation in cytokine receptor in X-linked SCID can result in what?

A

Decreased NK cells

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22
Q

One rare but for some reason important type of Autosomal Recessive (AR) SCID to…

A

missing JAK3

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23
Q

The most common AR SCID is due to …

A

ADA deficiency

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24
Q

Function deficiencies caused by AR SCID ADA deficiency are:

A

Decreased: T-cells, Serum IG AND B-cells

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25
Q

Deficiency in ADA enzymes causes accumulation of what toxic products/metabolites that affects DNA synthesis in proliferating cells? Lymphocytes are also very sensitive to these toxins.

A

dATP

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26
Q

Deficiency in PNP enzymes causes accumulation of what toxic products/metabolites that affects DNA synthesis in proliferating cells? Lymphocytes are also very sensitive to these toxins.

A

dGTP

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27
Q

AR SCID due to PNP deficiency causes what function defects?

A

Decreased T-cells ONLY

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28
Q

Ideal method for SCID treatment is

A

Stem Cell transplant (however must have compatible donor)

Side note for curious Jacob…less ideal but still viable would be gene therapy (insert gene = correct defect) but this has only really been successful in ADA type treatment

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29
Q

There has been concern about using viral vectors in gene therapy treatment b/c of the development of T-cell Leukemia in some patients with X-linked SCID patients. What is used now instead?

A

Lentivirus

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30
Q

Disease with X-linked B-cell maturation defect?

A

XLA (Bruton’s X-linked Agammaglobulinemia)

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31
Q

XLA is diagnosed at 5-6 months of age (later than SCID). This is because…

A

Maternal IgG protects baby for first 6 months of life. Unlike SCID, only B-cells are affected in XLA not T-cells.

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32
Q

Recurrent otitis media (note: not that uncommon in “normal” people too), PNEUMONIA (recurrent infections rare in normal healthy kid), and sinusitis (often chronic) are indicators of …

A

XLA

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33
Q

infectious agents that are most common in XLA patients are:

A

S.pneumonia or H.Flu (type b)

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34
Q

You will NOT see CD19 positive B cells in peripheral blood in what disorder?

A

XLA

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35
Q

You will see decreased serum IG, absence of plasma cells, increased autoimmune diseases, and underdeveloped germinal centers in LN, Peyer’s patches, appendix, and tonsils in patients with….

A

XLA

36
Q

True or False? In XLA T-cell reactions are within normal limit.

A

True. They are not fucked up at all. Remember this is a B-cell issue.

37
Q

CD19 is a marker for?

A

Mature B-cells

38
Q

DiGeorge Syndrome results from?

A

dysmorphogenesis of 3rd and 4th pharyngeal pouches

39
Q

3rd and 4th pharyngeal pouches give rise to what?

A
  • thymus and parathyroid (at 6-8 weeks)

- aortic arch and portions of lip and ears (at 12 weeks)

40
Q

Name so diagnostic features of DiGeorge Syndrome

A
  • Neonatal tetany
  • absent thymic shadow
  • decreased number of T-cells
  • decreased to absent antibody response (remember T-cells are needed for B-cells to function properly)
  • LN depletion of paracortical areas
41
Q

Just a random thing to know for DiGeorge

A

Note: thymic tissue, if found (fat chance), does contain Hassals corpuscles, normal density of thymocytes and corticomedullary distinction.

42
Q

DiGeorge Syndrome has a unique deletion found in 90% of diagnosed. The deleted region is responsible for development of brachial arch and great vessels. What is this deletion.

A

22q11 (spectrum of disorders resulting from small deletion of band q11.2 on long arm of chromosome 22)

43
Q

Name the primary immunodeficiency associated with defects in lymphocyte activation that is characterized by: Mutation in CD40 Ligand on X chromosome resulting in the absence of CD40L on CD4+ T-cells.

A

X-linked hyper IgM syndrome

44
Q

Lack of class switching and elevated IgM levels is associated with what syndrome?

A 
X-linked hyper IgM syndrome
Note: CD40L on T-cells cant bind to B-cells to induce class switching
45
Q

X-linked hyper IgM syndrome can cause defects in CMI (DTH) leaving patient fucked/susceptible to what intracellular microbe?

A

pneumocystis jiroveci

46
Q

Most common primary immunodeficiency?

A

Selective IgA defciency

47
Q

Why can’t you shoot up marijuana?

A

Because you will get a budclot

48
Q

Lil’ Timmy is for the most part asymptomatic, however recently he has had recurrent sinopulmonary infections and diarrhea. Upon a fullwork you notice his he has low serum IgA but normal levels of the other isotypes. What the hell is wrong with Lil’ Timmy?

A

He has SELECTIVE IgA DEFICIENCY

49
Q

Brent Smith just found out he has Common Variable Immunodeficiency. He is sad and his blood has low serum levels of what?

A
  • IgA and IgG (both low)

- IgM is normal to low

50
Q

Characterized by failure in maturation of B-cells into plasma cells.

A

Common Variable Immunodeficiency

51
Q

Major associated problems with ________ are respiratory and gastrointestinal infections with pyogenic bacteria. _______ also has highly variable presentations and pathogenesis.

A

Common Variable Immunodeficiency

52
Q

Mature B cells but absent plasma cells =

A

Common Variable Immunodeficiency

53
Q

Clinical Triad WISKOTT-ALDRICH SYNDROME (X-linked multisystem syndrome)

A

-lowered platelet count (thrombocytopenia)
-eczema/skin rashes
-recurrent bacterial infections (can’t respond to bacterial polysaccharides)
Note: jacob did you know that recently this has been treated with gene therapy?

54
Q

Wiskott-Aldrich Syndrome can also have low Ig__?

A

IgM

55
Q

Ataxia Telangiectasia: This disease is not primarily an immunodeficiency. True or False?

A

True

56
Q

Patient has low levels of IgA and IgG w/ a reduction in T-cells. Also patient has inability to respond to skin tests. What the hell is wrong?

A

Ataxia Telangiectasia

57
Q

What is the multisystem disorder responsible for the following symptoms:
Neurologic: staggering gate
Vascular: abnormal dilation
Immunogenic: Affects both B and T-cells

A

Ataxia Telangiectasia

58
Q

Defects in innate immunity are characterized by what ?

A

-recurrent intracellular bacterial and fungal infections

59
Q

An innate immunity defect characterized by defects in selectin ligand / beta chain of integrin

A

LAD (leukocyte adhesion deficiency)

60
Q

Bacterial infections w/ out pus formation and limited recruitment of cells to site of inflammation (ex: during a pyogenic infection) is seen in …

A

LAD

61
Q

A protein trafficking defect (autosomal recessive) characterized by impaired phagolysosome formation and giant granules in leukocytes (due to fusion of granules arising from Golgi).

A

Chediak-Higashi syndrome

62
Q

Patient presents with T-cell deficiency (lack of thymus); hypocalcemia (lack of parathyroid) and abnormalities of heart, great vessels, and face. What the hell is wrong?

A

DiGeorge Syndrome

63
Q

A primary autoimmune deficiency characterized by complete lack of immunoglobulin due to disordered B-cell maturation. Pre- and Pro- B-cells can not mature. What the hell is wrong?

A

XLA

-note: jacob…dont give these kids live vaccines.

64
Q

Mutation is WASP gene; X-linked

A

Wiskcott-Aldrich

65
Q

Phagocytes from patients have giant cytoplasmic granules; granules are dysfunctional. Phagocytes do not have ability to kill bacteria. Name the syndrome.

A

Chediak-Higashi Syndrome

66
Q

Chronic Granulomatous Disease (CGD)

A
  • Defective production of reactive oxygen intermediates (lack NADPH oxidase 02–>surpaoxide)
  • Intracellular survival of microbes results in granuloma formation
67
Q

Nitroblue tetrazolium test (NBT) measures oxidative burst and is used in diagnosing what disease?

A

CGD

68
Q

NBT dye turns blue if….

NBT dye remains yellow/ colorless if…

A 
blue= NADPH normal
colorless= NADPH absent (CGD)
69
Q

C5-C9 compliment deficiencies are at risk for …

A

Neisseria Infection (N gonorrhoeae and N meningitidis)

70
Q

C2 and C4 compliment deficiencies are at risk for …

A
  • increased incidence of immune complex diseases (resembling SLE)
  • Rheumatoid issues
71
Q

C1 and C3 deficiencies are at higher risk for…

A

infections with encapsulated organisms

72
Q

Compliment regulation defect example is

A

Hereditary angioedema

73
Q

Hereditary angioedema cause?

A

Deficiency if C1 inhibitor. Results in excessive C4 and C2 activation. Leads to localized edema / swelling (linked to increased levels C2 kinin–>swelling)

74
Q

Deficiency in DAF (Decay Accelerating Factor), host cells not protected from activation of compliment, characterized by intravascular hemolysis (hemolytic anemia) at night.

A

Paraoxsymal Nocturnal Hemoglubulinemia

75
Q

Secondary or Acquired immunodeficiencies are not genetic but rather they are acquired during life. An example is…

A

AIDs

76
Q

Acquired Immunodeficiencies linked to immunosuppression from another disease process include: name 3

A
  • protein calorie malnutrition
  • advanced widespread cancer
  • certain parasitic and viral infections (ex measles virus can infect lymphocytes)
77
Q

Herpes virus can secrete proteins similar to

A

IL-10

78
Q

Trypansoma cruzi is associated with reduced expression of ____ receptors on T-cell and thus can cause an acquired/ secondary immunodeficiency.

A

IL-2

79
Q

Secondary Immunodeficiencies linked to complications of therapy include:

A
  • drugs that kill or inactivate lymphocytes (anti-inflammatory and immunosuppressive drugs)
  • absence/surgical removal of spleen (decreased phagocytosis of microbes and increased susceptibility to encapsulated microbes.
80
Q

A self limited disorder characterized by a prolongation of physiologic hypogammaglobulinemia (usually prolonged to 9-15 months of age but may take 2-4 years to become normal)

A

Transient Hypogammaglobulinemia of Infancy (THI)

81
Q

Maternal derived IgG declines after birth and disappears by 3-6 months. At this time infant’s production of IgG is not fully developed. The neonate depends significantly on _______ immunity.

A

innate

82
Q

Maternal derived IgG declines after birth and disappears by 3-6 months. At this time infant’s production of IgG is not fully developed. Overall function of neonate immune system is reduced. Development of Th1 and CD8+ T-cells also delayed. Baby is most susceptible to infection during 6 mo to one year. Name the term.

A

Physiologic Hypogammaglobulinemia

83
Q

Immunosenescense

A

refers to the changes in immunity with increasing age

84
Q

Features of immunosenescense: (GULI)

A
  • Greater propensity for autoimmune response
  • Unsustained memory response
  • Lingering low grade inflammation
  • Impaired ability to respond to new antigens
85
Q

Clinical Consequences of immunosenescense:

A

Cancer, increased infections, and autoimmune diesease

86
Q

Prevention or compensation for age-related immunological defects are needed for healthy aging. True or false?

A

True

87
Q

Brent’s sister is hotter than Jacob’s mom. True or False.

A

im into bleached buttholes… I have to give the edge to brents sister…but both are solid first round draft picks.

POD Exam 3 (8 decks)

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