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IMSE LEC > MIDTERM - immunodeficiencies and immunoproliferative disorders > Flashcards

MIDTERM - immunodeficiencies and immunoproliferative disorders Flashcards

1
Q

Deficiencies of the immune system is also known as

A

immunodeficiencies

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2
Q

Deficiencies of the immune system include disorders of _

A

phagocytic cells.
B lymphocytes,
a combination of T and B lymphocytes,
the complement system application of
disinfectant.

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3
Q

The mechanisms of immunodeficiencies it could
be ____

A

acquired or congenital

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4
Q

____ result in a decreased ability
to phagocytize and kill bacteria.

A

Phagocytic cell deficiencies

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5
Q

is a genetic disease characterized by ineffective
killing of bacteria by neutrophils.

A

Chronic Granulomatous Disease (CGD)

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6
Q

characterized by inability of
phagocytes to make the NADH oxidase

A

Chronic Granulomatous Disease (CGD)

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7
Q

the important enzyme in generating the hydrogen peroxide

A

NADH oxidase

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8
Q

It is used
to kill the ingested bacteria.

A

Hydrogen peroxide

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9
Q

The effect of ___ is in line with the
decrease production of the hydrogen
peroxide

A

CDG

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10
Q

CGD is caused by a defect in __, which results in decreased hydrogen peroxide
production.

A

cytochrome b

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11
Q

___ is necessary for
producing the toxic superoxides that are critical in bacterial killing.

A

Hydrogen peroxide

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12
Q

Diagnosis of CDG

A

nitroblue tetrazolium (NBT) reductase test

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13
Q

is used to detect impaired
neutrophil phagocytosis.

A

nitroblue tetrazolium
(NBT) reductase test

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14
Q

mechanism of nitroblue tetrazolium reductase test

A

The neutrophils of CGD patients fail to reduce the Nitroblue tetrazolium dye

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15
Q

Symptoms of chronic granulomatous disease (CGD)

A

suffer from recurrent infections caused by catalase-positive bacteria, yeast, and fungi.

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16
Q

Treatment for CGD includes

A

use of GM-CSF or G
CSF and IFN-y.

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17
Q

is inherited as an autosomal
recessive trait and is one of the most common
inherited disorders.

A

MPO DEFICIENCY

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18
Q

MPO stands for

A

myeloperoxidase
deficiency”

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19
Q

have decreased or absent in
the primary granules of the
neutrophils

A

MPO deficiency

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20
Q

In mpo deficiency

The __ in the primary granules of
neutrophils is decreased or absent, and
although phagocytosis takes place
normally, bacterial killing is INEFFICIENT.

A

MPO

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21
Q

In MPO deficiency, which one is more impaired than bacterial killing

A

fungal killing

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22
Q

healthy patients with MPO
deficiency do not have an increased
frequency of infection, DIABETIC PATIENTS
who have this disorder may have an
increase in ___. infections.

A

Candida spp

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23
Q

is another inherited disorder in which
the AEROBIC SYSTEM of neutrophils is impaired.

A

Glucose-6-Phospate Dehydrogenase (G6PD)
deficiency

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24
Q

This deficiency results in a SUBSTANTIAL decrease in the amount of hydrogen peroxide produced during phagocytosis, and thus decreased bacterial killing efficiency

A

Glucose-6-Phospate Dehydrogenase (G6PD)
deficiency

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25
Q

___ is considered as an inherited
disorders their mechanism is impaired
aerobic system of the neutrophils.

A

G6PD

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26
Q

is a rare,
autosomal recessive trait characterized by a
decrease or absence of specific complement
component receptors on neutrophils, monocytes
and lymphocytes.

A

CR3 (iC3b receptor) Deficiency

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27
Q

CR3 (iC3b receptor) are
responsible for ___

A

adherence-related functions

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28
Q

CR3 (iC3b receptor) Deficiency result in

A

defective margination and
diapedesis of neutrophils,

impaired chemotaxis,

ineffective phagocytosis.

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29
Q

this disorder will make T lymphocytes
adhere poorly to target cells

A

CR3 (iC3b receptor) Deficiency

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30
Q

effect of cr3 deficiency

A

Clinically, there is
an increased frequency of bacterial infections,
a decreased inflammatory response, and
neutrophilia.

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31
Q

is inherited as an
autosomal recessive trait. Neutrophils fail to
develop specific granules during myelopoiesis,
and as a result, patients who have this disorder
experience severe recurrent bacterial infections

A

Specific Granule Deficiency

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32
Q

One of the most common examples of
this would be the Chediak-Higashi
syndrome.

A

Specific Granule Deficiency

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33
Q

under the specific granule deficiency

is an inherited
disorder that is characterized by the abnormal
fusion of primary granules in neutrophils

A

Chédiak-Higashi syndrome

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34
Q

During phagocytosis, degranulation is
impaired, and little or no MPO is
released into the phagosome.

A

Chédiak-Higashi syndrome

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35
Q

Patients who have ____have recurrent bacterial
infections and are also characterized by
ALBINISM AND EXTREME PHOTOSENSITIVITY

A

Chédiak-Higashi
syndrome

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36
Q

conditions under lazy leukocyte syndrome

A

job syndrome
tuftsin deficiency
actin dysfunction

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37
Q

also known as
hyperimmunoglobulin E

A

Job syndrome

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38
Q

is
characterized by poor chemotaxis and
recurrent skin infections and abscesses
or nana.

A

job syndrome

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39
Q

___ is a chemotaxin that also improve phagocyte
motility, engulfment and oxidative
metabolism

A

Tuftsin

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40
Q

A deficiency of the
cytoskeletal protein actin

A

Actin dysfunction

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41
Q

Actin dysfunction.

A deficiency of the
cytoskeletal protein actin, can result in
___ and ___.

A

decreased bacterial motility and
chemotaxis

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42
Q

may be
inherited or acquired and account for MORE THAN HALF of all immunodeficiencies.

A

B-lymphocyte Immunodeficiencies

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43
Q

➢ Second factor

A

B-lymphocyte Immunodeficiencies

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44
Q

A deficiency of a minor immunoglobulin,
such as ___, causes little if any increase
in the incidence of bacterial infections

A

IgD

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45
Q

because 75% to 85% of total
immunoglobulin is IgG, an individual
deficient in IgG would be significantly
affected.

true or false

A

true

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46
Q

is a sex-linked
disorder that primarily affects MEN. It is usually
recognized early in life when antibodies fail to
develop.

A

Bruton’s Agammaglobulinemia

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47
Q

in Bruton’s Agammaglobulinemia,
___cells may be found in the bone
marrow, but they do not mature

A

Pre-B cells

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48
Q

in bruton’s agammaglobulinemia

what globulin
levels are markedly decreased.

A

Gamma globulin

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49
Q

how to treat bruton’s agammaglobulinemia

A

This disorder may be treated with gamma
globulin preparations.

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50
Q

is
an acquired disorder in which one or two
immunoglobulin classes are deficient.

A

Common Variable Hypogammaglobulinemia

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51
Q

Total immunoglobulin levels are normal, because a decrease in one immunoglobulin is often
compensated by an increase in the production of
another.

A

Common Variable Hypogammaglobulinemia

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52
Q

___ deficiency is one of the
most common of Common Variable Hypogammaglobulinemia.

A

Selective IgA deficiency

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53
Q

is cause by the normal immaturity of the neonate’s immune system.

A

Neonatal Hypogammaglobulinemia

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54
Q

Neonatal Hypogammaglobulinemia

It corrects itself between the ages of ____ as infant’s immune
system matures.

A

6 and 12 months

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55
Q

composing only 7% of all immunodeficiencies.
These disorders may be acquired or inherited.

A

T-lymphocyte Immunodeficiencies

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56
Q

results when the thymus
gland develops abnormally during
embryogenesis.

A

DiGeorge syndrome

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57
Q

Abnormalities of other
endoderm-derived tissues are also seen

A

DiGeorge syndrome

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58
Q

DiGeorge syndrome

T lymphocytes are usually ___, but may be normal

A

decreased

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59
Q

DiGeorge syndrome

Most patients have high ___ratio.

A

CD4-CD8

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60
Q

In DiGeorge syndrome, the antibody responses are normal, which response is considered impaired?

A

cell mediated immune response

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61
Q

is an autosomal recessive
disorder. Patients are arrhythmic and are
especially susceptible to viral and fungal
infections, which can be fatal in these patients.

A

Nezelof syndrome

62
Q

an autosomal recessive, condition presents in infancy with recurrent or chronic pulmonary infections, oral or cutaneous candidiasis, diarrhea, skin infections,
urinary tract infections, and failure to thrive.

A

Purine Nucleoside Deficiency

63
Q

Purine Nucleoside Deficiency

Affects an enzyme involved in the
metabolism of ___

A

purines

64
Q

Produces a moderate to severe defect
in cell-mediated immunity with
normal or only mildly impaired humoral
immunity.

A

Purine Nucleoside Deficiency

65
Q

Purine Nucleoside Deficiency

Cells progressively decreases because
of the accumulation of ___, a toxic purine metabolite

A

deoxyguanosine triphosphate

66
Q

are the most serious of
the all immunodeficiencies that we have,
because both cell-mediated and humoral immune responses are affected.

A

Combined B- and T-lymphocyte
Immunodeficiencies

67
Q

are characterized by defects in Class I MHC antigen expression, Class II MHC antigen expression, or a combination of both.

A

Bare-Lymphocyte Syndromes

68
Q

in Bare-Lymphocyte Syndromes,

CD4-positive T lymphocytes are ___in number,
and B- and T-cell activation is ___.

A

decreased ; reduced

69
Q

may be inherited as autosomal recessive or X-linked traits.
All are characterized by markedly decreased numbers
of both T and B lymphocytes.

A

Severe Combined Immunodeficiency Disease

70
Q

is
caused by the human immunodeficiency virus 1 (HIV-1)
or the human immunodeficiency virus 2 (HIV-2).

A

Acquired Immunodeficiency Syndrome (AIDS)

71
Q

in patients with AIDS, the primary target cells are

A

The CD4-positive T lymphocytes; approximately 5% of B lymphocytes are also infected.

72
Q

_ is an X-linked recessive
disorder.

A

Wiskott-Aldrich Syndrome

73
Q

One of the clinical features involved with the Wiskott-Aldrich
Syndrome is the: __, __ , and ___

A

Triad of immunodeficiency,
eczema,
thrombocytopenia

74
Q

Deficiency of the naturally occurring antibodies to blood
group antigens

A

Wiskott-Aldrich Syndrome - WAS.

75
Q

Wiskott-Aldrich Syndrome, WAS.

(isohemagglutinins – ___ antibodies
against ABO blood group antigens)

A

IgM

76
Q
A
77
Q

in WAS or wiskot-aldrich syndrome

Have low levels of __, normal levels of __, and ___, and increased levels of __

A

IgM; IgA and IgG; IgE

78
Q

in wiskott-aldrich syndrome, there is as well an Abnormality of the integral membrane protein ___, which
is involved in the regulation of protein glycosylation.

A

CD43

79
Q

Rare autosomal recessive

A

Ataxia Telangiectasia

80
Q

is a rare autosomal
recessive/disorder in which it is also considered as a rare
childhood neurological disorder.

A

Ataxia Telangiectasia

81
Q

degeneration in the part of the brain causing problms with the motor movements and speech

A

Ataxia Telangiectasia

82
Q

Syndrome characterized by cerebellar ataxia and
telangiectasias, especially on the ___ and ___

A

earlobes and conjunctiva

83
Q

___ are small, widened blood vessel on the skin
or or so called spider veins.

It can be seen
especially on the earlobes and conjunctiva.

A

Telangiectasia

84
Q

spider veins are seen in

A

It can be seen
especially on the earlobes and conjunctiva.

85
Q

Abnormal genes produce a combined defect of both
humoral and cellular immunity.

A

Ataxia Telangiectasia

86
Q

describe the t cells of patients with ataxia telangectasias

A

Number of circulating T cells is often decreased

87
Q

Defect in a gene that is apparently essential to the
recombination process for genes in the immunoglobulin
superfamily

A

ataxia telangectasias

88
Q

Myeloproliferative disorder – consists of CML, MMM, PV, ET

CML MEANS

A

Chronic Myeloid Leukemia

89
Q

Myeloproliferative disorder – consists of CML, MMM, PV, ET
\

MMM?

A

Myelofibrosis with Myeloid Metaplasia

90
Q

pv means

A

Polycythemia Vera, an increase production of red
blood cells; it is also considered as a myeloproliferative
disorder.

91
Q

ET means

A

Essential Thrombocytopenia, a decrease production
of the thrombocytes or platelets.

92
Q

Classified as leukemia and lymphoma

A

Lymphoproliferative disorder

93
Q

__
are malignant cells. They are primarily present in the bone
marrow and in the peripheral blood.

A

Leukemias

94
Q

___ –
malignant cells din and it mainly arise in the lymphoid
tissue such as lmph nodes tonsils and splen

A

Lymphoma

95
Q

They are considered biologically distinct and not classified
as either leukemia or lymphoma

A

Plasma Cell Dyscrasias/Disorder:

96
Q

___is a premalignant condition, which is associated
as a Monoclonal Gammopathy of Undetermined
Significance (MGUS)

A

MGUS

97
Q

SMM –

A

Smoldering Multiple Myeloma

98
Q

LYMPHOMA is divded into

A

Hodgkin’s Lymphoma and Non-Hodgkin’s
Lymphoma.

99
Q

he lymphomas can be divided into Hodgkin’s Lymphoma
and Non-Hodgkin’s Lymphoma.

Among the two, and pinaka
kilala dito would be the ____

A

kin’s Lymphoma

100
Q

Highly treatable and often curable lymphoma that occurs both
in young adults and in the elderly.

A

Hodgkin’s Lymphoma

101
Q

a lymphoma that is It is characterized by the presence of Reed Sternberg (RS) cells
in affected lymph nodes and lymphoid organs

A

Hodgkin’s Lymphoma

102
Q

Though, itong RS cells ay pinagtalunan pa kung san ba
nanggagaling itong RS cells na ito. Through the different
studies, generally, nagmula itong ating RS cells in line with
a__

A

B cell lineage.

103
Q

___ are typically large with a bilobed nucleus and two
prominent nucleoli.

A

RS cells

104
Q

This gives the cell an owl’s eyes appearance.
Hodgkin’s Lymphoma

A

rs cells

105
Q

__ seems to be the greatest risk for the
Non-Hodgkin’s Lymphoma.

A

Immunosuppression

106
Q

__
Lymphoma has a wide range of neoplasm.

A

Non-Hodgkin’s

107
Q

in non hodkins, Over ___ of the patients are greater than 60 years of age and the
incidence is greater in men compared with the women.

A

two-thirds

108
Q

it has an increased risk also
for
some
autoimmune
diseases,
congenital
immunodeficiency disorders, organ transplantation, and
exposure to other infectious agents.

A

Non-Hodgkin’s Lymphoma

109
Q

leukemia

AML
(* ALL
* CML
* CLL

A

AML (Acute Myeloid Leukemia)
* ALL (Acute Lymphocytic/Lymphoblastic Leukemia)
* CML (Chronic Myelogenous Leukemia)
* CLL (Chronic Lymphocytic Leukemia/Lymphoma)

110
Q

classify leukemia based on
morphology and of course we should consider the
cytochemical staining

A

FAB Classification

111
Q

morphology, cytochemical staining, genetic
characteristics, serological markers (CD markers).

A

WHO criteria

112
Q

The World Health Organization (WHO) considered __ and ___a single disease with different clinical
presentations. They both reveal that they have the
capacity for the B cell marker.

A

CLL
and SLL

113
Q

__ is a common hematopoietic malignancy that involves
the expansion of a clone of B cells that have the appearance
of small mature lymphocytes.

A

CLL

114
Q

What are the B cell
marker that are mainly incorporated with the CLL and SLL? T

A

hey have both CD19, but weakly expression of
CD20.

115
Q

CLL primarily occurs in patients over __years of age.

A

45

116
Q

CLL is the Chronic Lymphocytic Leukemia. They have
revealed a B cell marker CD19, but weak expression of
CD20. Aside from having a small mature lymphocyte, CLL
is incorporated also in the __ cells

A

smudge

117
Q

__ CELLS are remnants of cells that lack any identifiable
cytoplasmic membrane or nuclear structure. S

A

SMUDGE

118
Q

Smudge cells, also
called ,

A

BASKET CELLS

119
Q

are most often associated with
abnormally fragile lymphocytes in disorders such as chronic
lymphocytic leukemia (CLL).

A

Smudge cells

120
Q

A rare, slowly progressive disease characterized by infiltration of
the bone marrow and spleen by leukemic cells, without
involvement of lymph nodes

A

HAIRY CELL LEUKEMIA

121
Q

hairy cell leukemia is seen in individuakls over __ years of age

A

20

122
Q

They often have irregular “hairy” cytoplasmic projections from
their surfaces.

A

HAIRY CELL LEUKEMIA

123
Q

HAIRY CELL LEUKEMIA

The malignant cells strongly express B-cell markers

A

CD19,
CD20, and CD22.

May weaker expression ito for the CD20.

124
Q

Includes
Multiple
myeloma
and
Waldenstrom
macroglobulinemia, MGUS and SMM.

A

PLASMA CELL DYSCARIAS

125
Q

MGUS means

A

Monoclonal Gammopathy of Undetermined
Significance

126
Q

SMM means

A

Smoldering Multiple Myeloma

127
Q

These conditions are characterized by the overproduction of a
single immunoglobulin component called a myeloma protein (M
protein), or paraprotein, by a clone of plasma cells.

A

PLASMA CELL DYSCARIAS

128
Q

these condition are characterized by a development of a
single immunoglobulin component and it is called as a
myeloma protein or M protein or paraprotein. It is by clone
of the plasma cells.

A

PLASMA CELL DYSCARIAS

129
Q

The laboratory evaluation is an important factor in line with
the diagnosis and differentiation of this condition.

A

PLASMA CELL DYSCARIAS

130
Q

Diagnosis and monitoring of the plasma cell dyscrasias
depend heavily on detecting and quantitating the __

A

M protein.

131
Q

A malignancy of mature plasma cells that accounts for
about 10 percent of all hematologic cancers.

A

MULTIPLE MYELOMA

132
Q

MULTIPLE MYELOMA

It is usually diagnosed in persons between ___
of age with a peak age of ____

A

40 and 70 years ; 67 years.

133
Q

The American Cancer Society estimates there are about
__ new cases of multiple myeloma diagnosed each
year in the United States, along with about __ deaths

A

20,000; 11,000

134
Q

Patients progress from asymptomatic MGUS to SMM to the
symptomatic disease multiple myeloma. ____% of people
with MGUS will progress to Multiple myeloma

A

20-25

135
Q

Patients with multiple myeloma typically have excess
___ in the bone marrow, a monoclonal
immunoglobulin component in the plasma and/or urine, and
lytic bone lesions.

A

plasma cells

136
Q

Malignant plasma cells phenotypically express

A

CD38,
CD56, and CD138.

137
Q

A malignant proliferation of IgM-producing lymphocytes and
corresponds to lymphoplasmacytoid lymphoma as defined
by the WHO.

A

WALDENSTROM MACROGLOBULINEMIA

138
Q

WALDENSTROM MACROGLOBULINEMIA

The median age of affected patients is ___

A

65 years

139
Q

The etiology of this disease is unknown

A

WALDENSTROM MACROGLOBULINEMIA

140
Q

Genetic factors are thought to be involved.

A

WALDENSTROM MACROGLOBULINEMIA

141
Q

The monoclonal IgM can accumulate in any tissue, forming
deposits that lead to inflammation and tissue damage.

A

WALDENSTROM MACROGLOBULINEMIA

142
Q

The median length of survival for patients with
Waldenström’s macroglobulinemia is longer than with
multiple myeloma—____

A

5 years versus 3 years.

143
Q

20-30% of patient the IgM paraprotein behaves as
_____ - precipitate at cold temp. and can occludes
small vessels in the extremities in cold weather.

A

cryoglobulin

144
Q

The laboratory is involved in three major ways in evaluating
lymphoproliferative disorders.

A

1st, it can assess the immunophenotype of
hematopoietic cells in the blood, bone marrow, or
lymphoid tissues by flow cytometry.
Done by
detecting the antigens on the surface of the cells that are characteristic of specific lineage and stage
of differentiation.

2nd, evaluating the amount and characteristics of
immunoglobulins.

3rd, genetic and chromosomal abnormalities
assessment.

145
Q

Analysis of cell surface marker expression is commonly
used in the diagnosis and classification of leukemias and
lymphomas.

A

MMUNOTYPING BY FLOW CYTOMETRY

146
Q

Samples of potentially neoplastic cells are incubated with
antibody preparations specific for relevant antigens.

A

IMMUNOTYPING BY FLOW CYTOMETRY

147
Q

n a malignant disorder, the clonal
proliferation of transformed plasma cells leads to
overproduction of a single immunoglobulin.

A

monoclonal
gammopathy.

148
Q

s a technique in which serum proteins are separated on the
basis of their size and electrical charge.

A

SERUM PROTEIN ELECTROPHORESIS

149
Q

SPE results in four regions:

A

Albumin, and Alpha, Beta, and
gamma globulins.

150
Q
A

IMSE LEC (15 decks)

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