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PPS - From Nelson's > Immunology > Flashcards

Immunology Flashcards

1
Q 
1yr old female presented with recurrent staphylococcal infection
What test will you request? 
A. HIV Elisa
B. C3 C4 assay
C. Nitroblue tetrazolium
D. Quantitative immunoglobulin assay
A

C

Phagocytic defect

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2
Q 
7 month old male presenting as failure to thrive, recurrent diarrhea and pneumonia. Patient came in for pcap, on culture noted H. Influenza
What do you suspect?
A. Combined immunodeficiency
B. T cell immunodeficiency
C. Wiskott Aldrich
D. B cell immunodeficiency
A

D.

Loss of antibody mediated opsonization for encapsulated pyogenic bacteria

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3
Q

most common b cell defect

what to screen?

A

selective igA deficiency

igA levels

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4
Q

one of most useful test to check b cell function

A

isohemagglutinins (check antibodies to type A or B blood)

measures mostly igM

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5
Q

common infection in x linked agammaglobinemia

A

enteroviruses

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6
Q

common problems in CVID combined variable immunodeficiency

A

autoimmune disease

lypmhoid hyperplasia

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7
Q

most cost effective test for T cell function

A

candida skin test

positive skin test rules out T cell defect

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8
Q

when to suspect phagocytice cell dysfunction

A

recurrent staph infections

or gram negative infections

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9
Q

what diagnostic for phagocytice cell dysfunction

A

neutrophil respiratory burst after phorbol ester
using rhodamine dye
previously nitroblue tetrazolium used

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10
Q

most effect lab test for screening complement defect

A

CH50 assay

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11
Q

severe hypoagammaglobulinemia
abscence of circulating b cells
small or absent tonsils
no palpable lymph nodes

A

x linked agammaglobulinemia

OR bruton agammaglobulinemia

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12
Q

drugs associated with producing CVID

A

phenytoin
pencillamine
gold
sulfasalazine

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13
Q

immunodeficiency with inadequate response to EBV infection

A

x linked lymphoproliferative disease

Duncan disease

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14
Q

therapy for b cell defect except for mutation in cd40 ligand

A

IVIG to provide missing antibodies and not increase igG levels
cd40 ligand mutation treated with stem cell transplant

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15
Q

caution with ___ use in patients with igA deficiency

A

IVIG or blood products

might produce anaphylaxis

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16
Q

thymic hypoplasia results from

A

dysmorphogenesis of 3rd and 4th pharyngeal pouch

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17
Q

catch 22

A 
digeorge
chromosome q2211.2
cardiac
abnormal facies
thymic hypoplasia
cleft palate
hypocalcemia
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18
Q

one third of kids with digeorge have __ association

A 
CHARGE
coloboma
heart defect
atresia choanae
retardation
ear anomalies/deafness
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19
Q

digeorge prone to

A

fungi
viruses
pneumocystis jiroveci
graft versus host disease (blood transfusion)

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20
Q

SCID prone to

A

fungi
viruses
pneumocystis jiroveci
graft versus host disease (blood transfusion)

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21
Q

most common form of scid in usa

A

x linked scid
low T and NK
high B cells

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22
Q

second most common form of scid

A

autosomal recessive scid

absence of adenosine deaminase (ADA)

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23
Q

how to distinguish cid from scid

A

cid has low but not absent t cell function
survive longer than scid
serum immunoglobulins may be normal or elevated

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24
Q

x linked recessive
atopic dermatitis
thrombocytopenic purpura with normal appearing megakaryocytes but small defective platelets
xp11.22
prolonged bleeding at infancy
prone to infection with bacteria with polysaccharide capsules like strep. pneumoniae

A

wiskott aldrich

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25
Q

wiskott aldrich lab findings

A

impaired humoral immunity
igG normal or low
low isohemagglutinins, low igM, elevated igA igE,

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26
Q

tx for wiskott aldrich

A

ivig

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27
Q

immunodeficiency with immunologic, neurologi, endocrinologic, hepatic and cutaneous abnormalities

A

ataxia telangiectasia

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28
Q

features of ataxia telangiectasia

A

progressive cerebellar ataxia, oculocutaneous telangiectasias, chronic sinopulmonary disease, a high incidence of malignancy, and variable humoral and cellular immunodeficiency

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29
Q

Abnormal lymphocyte apoptosis leading to polyclonal population of t cells
Do not express cd4 and cd8

A

Autoimmune lymphoproliferative syndrome

Canale Smith syndrome

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30
Q

Lymphadenopathy
Hepatosplenomegaly
Hypergammaglobulinemia igG igA
Hemolytic anemia

A

Autoimmune lymphoproliferative syndrome

Canale Smith syndrome

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31
Q

Autoimmune lymphoproliferative syndrome
Canale Smith syndrome
Common malignancies

A

Hodgkin
Non Hodgkin lymphoma
Tumor of thyroid skin heart lung

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32
Q

Treatment of
Autoimmune lymphoproliferative syndrome
Canale Smith syndrome

A

Corticosteroids

Immunosuppressant like cyclophosphamide methotrexate azathioprine

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33
Q

First weeks of life watery diarrhea (autoimmune enteropathy)
Eczematous rash
Diabetes
Hyperthyroidism or hypo
Coomb’s positive hemolytic anemia thrombocytopenia neutropenia

A

Immune dysregulation polyendocrinopathy x linked syndrome

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34
Q

Tx Immune dysregulation polyendocrinopathy x linked syndrome

A

Cyclosporine tacrolimus sirolimus

Steroids

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35
Q

Part of phagocytic system

A

Granulocytes:
Neut
Eos
Baso

Mononuclear phagocytes:
Monocytes
Tissue macrophages

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36
Q

Principal sites of macrophages in tissues

A

Liver (Kupffer cells)
Lung (interstitial and alveolar macrophages)
Connective tissue, adipose tissue, and interstitium of major organs and skin
Serosal cavities (pleural and peritoneal macrophages)
Synovial membrane (type A synoviocytes)
Bone (osteoclasts)
Brain and retina (microglial cells)
Spleen, lymph nodes, bone marrow Intestinal wall
Breast milk
Placenta Granulomas (multinucleated giant cells)

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37
Q

Function of macrophages

A
  1. phagocytosis
  2. presentation of antigens to lymphocytes
  3. enhancement or suppression of the immune response through release of a variety of potent hormone-like factors termed cytokines
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38
Q

Tx in chronic granulomatous disease

A

Ifn gamma

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39
Q

Role macrophages in gaucher’s

A

accumulation of cell debris that is normally cleared

enzyme glucocerebrosidase functions abnormally, thus allowing accumulation of glucocerebroside from cell membranes in Gaucher cells throughout the body

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40
Q

Diseases where macrophage activation is pathologically excessive

A

Familial and acquired hemophagocytic lymphohistiocytosis

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41
Q

uncontrolled activation of T cells and macrophages, with resultant fever, hepatosplenomegaly, lymphadenopathy, pancytopenia, marked elevation of serum proinflammatory cytokines, and macrophage hemophagocytosis

A

Familial and acquired hemophagocytic lymphohistiocytosis

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42
Q

malignancy-like overgrowth of Langerhans-type DCs

A

Langerhans cell histiocytosis

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43
Q

Tx for patients with hypereosinophilic syndrome

A

Steroids

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44
Q

Tx for patients with hypereosinophilic syndrome

A

Steroids

45
Q

Life threatening condition associated with eosinophilia

A

Lofflers endocarditis

46
Q

Eosinophilia associated with primary immunodeficiency

A

Wiskott Aldrich
Omenn syndrome
Hyperimmunoglobulin e syndrome

47
Q

Children with phagocytic defects present

A

with deep tissue infection, pneumonia, adenitis, or osteomyelitis rather than blood stream infections

48
Q

Difference between the three leukocyte adhesion defects

A

LAD 1 mutation in cd11/cd18 serious softi tissue and skin infection

LAD 2 normal cd11/cd18 neurologic defects, cranial facial dysmorphism, and absence of the erythrocyte ABO blood group antigen (Bombay phenotype)

LAD 3 Glanzmann thrombasthenia-like bleeding disorder, delayed separation of the umbilical cord

49
Q

autosomal recessive disorder characterized by increased susceptibility to infection
caused by defective degranulation of neutrophils, a mild bleeding diathesis,
partial oculocutaneous albinism, progressive peripheral neuropathy, and a tendency to develop a life-threatening form of
hemophagocytic lymphohistiocytosis

A

Chédiak-Higashi syndrome

50
Q

most life-threatening complication of chediak higashi
is the development of an accelerated phase characterized by pancytopenia, high fever, and lymphohistiocytic infiltration of liver, spleen, and lymph nodes

A

hemophagocytic lymphohistiocytosis

51
Q

Treatment for chediak higashi syndrome

A

Vitamin c

Stem cell

52
Q

most common inherited disorders of phagocytes

Autosomal recessive

A

Myeloperoxidase (MPO) deficiency

53
Q

neutrophils and monocytes capable of normal chemotaxis, ingestion, and degranulation

but unable to kill catalase positive microorganisms because of a defect in the generation of microbicidal oxygen metabolites

A

Chronic granulomatous disease

54
Q

Chronic granulomatous disease phagocytic vacuoles lack microbicidal reactive oxygen species and remain ___

A

Acidic

55
Q

Primary lymphoid organs develop

A

Thymus and bone develop middle of first trimester

56
Q

Secondary lymphoid organs

A

—spleen, lymph nodes, tonsils, Peyer patches, and lamina propria

57
Q

Positive selection

A

Occurs through the interaction of immature thymocytes, which express low levels of TCR, with major histocompatibility complex (MHC) antigens present on cortical thymic epithelial cells

thymocytes with TCR capable of interacting with foreign antigens presented on self human leukocyte antigen (HLA) molecules are activated and develop to maturity

58
Q

Negative selection

A

occurs next in the medulla and is mediated by interaction of the surviving thymocytes

interaction mediates apoptosis (programmed cell death) of such autoreactive thymocytes

59
Q

swirls of terminally differentiated medullary epithelial cells
first seen in the thymic medulla at 16-18 wk of embryonic life

A

Hassall’s corpuscles (bodies)

60
Q

B-cell development begins in the fetal liver before__ of gestation

A

7wk

61
Q

NK cell activity is found in human fetal liver cells at __ wk of gestation

A

8-11

62
Q

Difference between class I and II MHC

A

Class I MHC molecules are found on most nucleated cells in the body

Class II MHC molecules are found on antigen-presenting cells (APCs), which include macrophages, dendritic cells, and B cells

63
Q

CD4 molecule binds directly to

A

MHC II on Antigen presenting cells

64
Q

CD8 on cytotoxic T cells binds

A

MHC class I molecule on the target cell

65
Q

primary antibody response

A

native antigen is carried to a lymph node draining the site, taken up by specialized cells called follicular dendritic cells (FDCs), and expressed on their surfaces

66
Q

secondary antibody response

A

occurs when these memory B cells again encounter that antigen

67
Q

Newborn infants have increased susceptibility to infections with Gram-negative organisms

A

because IgM antibodies, which are heatstable opsonins, do not cross the placenta

68
Q

IgM level of newborns rise by

A

6 days

69
Q

Serum igA detected at ___ for newborns

A

13th day

IgA does not Cross placenta

70
Q

Normal infants cannot usually produce antibodies to polysaccharide antigens until after __ yr of age

A

2 years old

Can produce earlier if the polysaccharide is conjugated to a protein carrier, as is the case for the conjugate Haemophilus influenzae type b and Streptococcus pneumoniae vaccines.

71
Q

thymus is largest relative to body size during

A

fetal life and at birth is ordinarily two-thirds of its mature weight, which it attains during the 1st yr of life.

72
Q

Thymus reaches its peak mass at

A

just before puberty, and then gradually involutes thereafter.

73
Q

T or f

By 1 yr of age, all lymphoid structures are mature histologically.

A

True

74
Q

Absolute lymphocyte counts in the peripheral blood also reach a peak during the

A

1st yr of life

75
Q

Viruses that cause neutropenia

A

influenzas A and B, adeno-virus, respiratory syncytial virus, enteroviruses, human herpes virus 6, measles, rubella, and varicella

76
Q

Nutritional cause of neutropenia

A

Vit b12 or folate deficiencies
Malnutrition
Low protein
Anorexia nervousa

77
Q

Cyclic neutropenia occurs

A

Every 21 days +-4 days

78
Q

Diagnosis of cyclic neutropenia

A

Get CBC 3x per week for 6-8 weeks

79
Q

What’s defect in cyclic neutropenia

A

Neutrophil elastase

Autosomal dominant

80
Q

Tx for cyclic neutropenia

A

Filgrastim

81
Q

Neuro deficit and sever neutropenia
Hax1 problem
Recessive form of the disease

A

Kostmann disease

82
Q

autosomal recessive disorder classically characterized by
neutropenia
pancreatic insufficiency
short stature with skeletal abnormalities

steatorrhea and failure to thrive because of malabsorption

frequent otitis media, pneumonia and eczema

A

Shwachman-Diamond syndrome (SDS)

83
Q

disorder of telomerase activity
bone marrow failure rather than isolated neutropenia

classical phenotype:
nail dystrophy, leukoplakia, mal-formed teeth, and reticulated hyperpigmentation of the skin

A

Dyskeratosis congenita

84
Q 
neutropenia
Partial albinism
 high risk of HLH
but peripheral blood granulocytes do not show giant granules 
 hypogammaglobulinemia
 caused by mutations in RAB27a
A

Griscelli syndrome type II

85
Q

Leukemoid reaction

A

<50,000

Mostly neutrophils

86
Q

Role of monocytosis in patients ongoing chemo

A

harbinger of the return of the neutrophil count to normal

87
Q

Classical pathway:

A

C1q, C1r, C1s, C4, C2, C3

88
Q

Alternative pathway:

A

Factor B, Factor D

89
Q

Membrane attack complex:

A

C5, C6, C7, C8, C9

90
Q

have SLE, but recurrent meningococcal infections are much more likely to be the major problem

A

C5, C6, C7, or C8 deficiency

91
Q

recurrent severe staphylococcal abscesses of the skin, lungs, and other sites and markedly elevated levels of serum IgE
candida is second most common pathogen
autosomal dominant

A

hyper igE syndrome

92
Q

lab findings in hyper igE syndrome

A

high igE
eosinophilia
normal T B NK cells
normal igG igA igM

93
Q

tx of hyper iG syndrome

A

ivig

94
Q

most common indication for HSCT in childhood

A

ALL

95
Q

autosomal recessive disease characterized by spontaneous chromosomal fragility,

which is increased after exposure of peripheral blood lymphocytes to DNA crosslinking agents, including clastogenic compounds, such as diepoxybutane, mitomycin C, and melphalan

A

Fanconi anemia

96
Q

main indications for performing HSCT in patients with sickle cell disease are

A

history of strokes, magnetic resonance imaging of central nervous system lesions associated with impaired neuropsychologic function, failure to respond to hydroxyurea

97
Q

major cause of mortality and morbidity after allogeneic hemato- poietic stem cell transplantation (HSCT)

A

graft -versus-host disease (GVHD)

caused by engraftment of immunocompetent donor T lymphocytes in an immunologically compromised host which show histocompatibility differences with the donor

98
Q

tx of GVHD

A

cyclosporine or tacrolimus or com- binations of either with methotrexate or prednisone, anti–T-cell anti- bodies, mycophenolate mofetil

99
Q

features of GVHD

A

primary manifestations are an erythematous maculopapular rash, persistent anorexia, vomiting and/or diarrhea, and liver disease with increased serum levels of bilirubin, alanine aminotransferase, aspartate aminotransferase, and alkaline phosphatase
thinning of hair
GI ulcers

100
Q

major cause of nonrelapse mortality and morbidity in long-term HSCT survivors

A

Chronic GVHD

101
Q

factor that confers favorable course in GVHD

A

skin manifestation only

102
Q

primary graft failure

A

failure to achieve a neutrophil count of 0.2 × 109/L by 21 days posttransplantation

103
Q

Secondary graft failure

A

loss of peripheral blood counts follow- ing initial transient engra ment of donor cells

104
Q

on pulmo ct of patient with progressing pneumonia noted enlarging nodule, the dense central core of infarcted tissue becomes surrounded by edema or hemorrhage, forming a hazy rim
what is sign?
dx?

A

halo sign

aspergillosis

105
Q

in aspergillosis, pulmonary infarcted central core cavitates, creating the __

A

crescent sign

106
Q

remains the most common and potentially severe viral complication in patients given allogeneic HSCT

A

CMV

107
Q

diarrhea, failure to thrive, generalized scaling rash, recurrent cutaneous/systemic infection

A

severe combined immunodeficiency syndrome

108
Q 
x linked recessive
thrombocytopenia
immune defect
recurrent severe bacterial infection
eczema
A

wiskott aldrich syndrome

109
Q

elevated igE
recurrent deep seated bacterial infection
chronic dermatitis
refractory dermatophytosis

A

hyper igE syndrome

PPS - From Nelson's (24 decks)

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