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Carpenter > Immunodeficiency > Flashcards

Immunodeficiency Flashcards

1
Q

what happens with all types of immunodeficiencies?

A

increased susceptibility to newly acquired infections
reactivation of latent infections
increased incidence of cancers

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2
Q

what immunodefiencies have defects in lymphocyte maturation

A

SCID
XLA
DiGeorge Syndrome

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3
Q

what immunodefiencies have defects in lymphocyte activation and function

A

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

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4
Q

what immunodefiencies have defects in innate immunity

A

Chronic granulomatous disease
Leukocyte adhesion deficiency
Complement deficiencies
Chediak-higashi syndrome

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5
Q

where are b cells in lymphnode

A

germinal center

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6
Q

where are T-cells in lymphnode

A

paracortical area

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7
Q

when you have a B-cell problem what type of infections do you see

A

pyogenic bacterial, enteric bacterial, viral

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8
Q

when you have a T-cell problem what type of infections do you see

A

viral and other intracellular microbial infections (atypical, fungi)

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9
Q

when you have a B-cell problem what histopathology and lab abnormalities do you see

A

Absent or reduced follicles and germinal centers in lymphoid organs
Reduced Ig serum levels

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10
Q

when you have a T-cell problem what histopathology and lab abnormalities do you see

A

reduced paracortical zone in lymphoid organs

Reduced delayed type hypersensitivity

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11
Q

list some clues to immunodeficiency in a patient

A 
infections that are: severe, recurrent, persistent 
weight loss and/or poor growth
family history 
swollen lymphoid organs
Autoimmune disorders (RA, SLE)
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12
Q

what is the defect in XSCID

A

mutation in common gamma chain receptor for cytokines (IL-2, 4, 7, 9, 11, 15, 21)

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13
Q

what functional deficiencies are seen in XSCID

A

decreased T cells (IL-7)
Bcell count normal but functional ability is down
Serum Ig decreased
NK cells decreased (IL-15)

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14
Q

What are two major AR SCID and what is most common

A 
ADA deficiency (most common)
PNP deficiency
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15
Q

Mechanism of action of AR SCID due to ADA OR PNP

A

Accumulation toxic metabolites in lymph

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16
Q

what functional deficiencies are seen in AR-SCID (due to ADA)

A

T-cell, B-cell, and serum Ig all decreased

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17
Q

what functional deficiencies are seen in AR-SCID (due to PNP)

A

T-cells decreased

B-cells and Serum Ig within normal limits

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18
Q

How is SCID treated?

A

bubble boy
stem cell transplant (ideal)
gene therapy (good with ADA)

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19
Q

Gene therapy in XSCID is done how

A

normal gamma gene introduced in bone marrow stem cells-lentivirus used to transfer vector

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20
Q

what is used to transfer vector in XSCID gene therapy

A

lentivirus

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21
Q

X-linked Agammaglobulinemia (XLA) defect

A

defect in Bruton tyrosine kinase- inability of pre-B cells to develop into mature B cells

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22
Q

when is XLA diagnosed and why

A

usually at 5-6 months because they have placental IgG at that time

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23
Q

how is XLA treated

A

gamma globulin injections

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24
Q

what sequelae does one normally see with XLA

A

otitis media
pneumonia
sinusitis
infectious with S. pneumonia, H. flu, staph

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25
Q

What test and for what is done for XLA

A 
no CD19 B-cells (checked via flow cytometry)
Low Ig levels
Germinal Centers depleted
Plasma cells absent
Increase in autoimmune disease
26
Q

DiGeorge Syndrome results from what

A

dysmorphogenesis of the 3rd and 4th pharyngeal pouches (thymus and parathyroid)

27
Q

Clinical presentation of DiGeorge Syndrome

A 
neonatal tetany
absence of thymic shadow on 
facial abnormalities (small jaw, lowest ears)
28
Q

DiGeorge is now considered a component of what chromosomal deletion (as it is found in more than 90% of patients with DiGeorge)

A

22q11.2

29
Q

X-linked Hyper IgM Syndrome is caused by what

A

mutation in CD40-L gene on X-chromosme (thus no CD40-L on T-cell)

30
Q

The mutation in X-linked hyper IgM syndrome leads to what

A 
absence of CD40-L on T-cell and lack of class switching of Ig
Increased IgM b/c it can't switch to other classes
31
Q

Selective IgA Deficiency is characterized by what

A

low serum IgA but normal levels of other isotypes

32
Q

what is the most common primary immunodeficiency?

A

Selective IgA Deficiency

33
Q

Common Variable Immunodeficiency is characterized by failure of what

A

maturation of B cells into plasma cells

34
Q

what is seen in patients with Common Variable Immunodeficiency

A

low serum levels of IgG and IgA, normal to low IgM
Have mature B cells
No plasma cells

35
Q

patients with Common variable Immunodeficiency are susceptible to what

A

B-cell problem so- bacterial infections (respiratory mainly) and pyogenic GI infections

36
Q

what infections would one see in both XLA and CVID

A

bacterial, enterovirus, Giardia infections

37
Q

Wiskott-Aldrich Syndrome is associated with what clinical problems

A

lowered platelet count
skin rashes (eczema)
recurrent bacterial infections

38
Q

Ataxia Telangiectasia is associated with what clinical signs/symptoms

A 
neurologic symptoms-staggering gate
vascular problems- spider veins
T and B cell defects
Low IgA and IgG levels
No normal DTH
39
Q

Leukocyte Adhesion Deficiency is a defect in what

A

adhesion of leukocytes to vascular endothelium via defects in selecting and beta-chain of integrin

40
Q

bacterial infections without puss are seen in what

A

Leukocyte Adhesion Deficiency

41
Q

Chediak-Higashi Sydrome is a disorder in what

A

phagocytes from patients have giant cytoplasm granules- but granules are dysfunctional

42
Q

Chronic Granulomatous Disease is a defect in what

A

production of reactive oxygen intermdiates- allows for intracellular survival of microbe

43
Q

intracellular survival of microbe results in what

A

granuloma formation

44
Q

Nitroblue tetrazolium test (NBT) is used to test for what and how

A

Chronic granulomatous disease- originally yellow and it turns blue/purple if ROS are produced

45
Q

neutrophil function test is used to test for what and how

A

chronic granulomatous disease- molecule gets oxidized to green fluorescent compound by NADPH oxidase

46
Q

C1 and C3 complement deficiencies are associated with what type of infections

A

encapsulated organisms

47
Q

C2 and C4 complement deficiencies are associated with what

A

increased incidence of immune complex diseases

48
Q

C5, 6, 7, 8, 9 complement defects are associated with what

A

Neisseria infections

49
Q

Hereditary Angioedema patients can present with what

A

acute swelling (localized edema) in a variety of anatomical locations- can look like anaphylaxis

50
Q

Hereditary Angioedema is due to what

A

C1 inhibitors deficiency- resulting in excessive C4 and C2 activation

51
Q

Hereditary Angioedema increased levels of what lead to localized edema

A

C2 kinin

52
Q

Paroxysmal Nocturnal Hemoglobulinemia is a deficiency in what

A

decay accelerating factor-host cells not protected from activation of complement

53
Q

Paroxysmal Nocturnal Hemoglobulinemia is characterized by what

A

characterized by intravascular hemolysis

54
Q

Secondary Immunosuppression can be due to what

A

protein-calorie malnutrition
advanced widespread cancer
certain parasitic and viral infections

55
Q

what virus can infect lymphocytes

A

measles

56
Q

what drugs kill or inactivate lymphocytes

A

anti-inflammatory drugs
immunosuppressive drugs
re

57
Q

absence of spleen leads to secondary immunodeficiency how?

A

decreased phagocytosis of microbes

increased susceptibility to encapsulated bacteria

58
Q

what is physiological hypogammaglobulinemia

A

maternal IgG declines to allow at 3-6 months of age but infants own IgG production is not fully developed at this time

59
Q

what is Transient Hypogammaglobulinemia of Infancy (THI)

A

prolongation of physiologic hypogammaglobulinemia

60
Q

How is THI diagnosed

A

IgG levels at least 2 standard deviations below the mean for aged-matched controls

61
Q

immunosenescence refers to what

A

changes in immunity with increasing age

Carpenter (1 decks)

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