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HEMA311 > Leukocyte Disorders > Flashcards

Leukocyte Disorders Flashcards

(100 cards)

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1
Q

what is the normal relative neutrophil count?

A

50-70%

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2
Q

determined the number of segmented and band neutrophils

A

absolute neutrophil count

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3
Q

nonpathologic causes of neutrophilia

A

strenuous exercise, emotional stress, shock, burns, trauma, labor, pregnancy, increase in epinephrine

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4
Q

reactive leukocytosis above 50x10^9/L with neutrophilia and a marked left shift (presence of immature forms)

A

leukemoid reaction

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5
Q

what is neutrophilic leukemoid reaction may be cocnfused as?

A

chronic myelogenous leukemia

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6
Q

presence of immature neutrophils, nucleated red blood cells. and teardrop RBCs in the same sample. may be accompanied by neutrophilia but not always

A

leukoerythroblastic reaction

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7
Q

what does leukoerythroblastic reaction point to?

A

space-occupying lesion in the bone marrow

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8
Q

sever form of neutropenia

A

agranulocytosis

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9
Q

maternal IgG crosses the placenta and binds to neutrophil-specific antigens inherited from the father

A

alloimmune neonatal neutropenia

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10
Q

a primary illness in children in which moderate to severe neutropenia develops as a result of antibodies to HNA-1

A

autoimmune neutropenia

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11
Q

autosomal recessive disorder characterized by marrow failure, pancreatic insufficiency, and skeletal abnormalities. intermittent neutropenia that fluctuates from severely low to near normal

A

Shwachman-Diamond syndrome

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12
Q

autosomal recessive disease characterized by severe neutropenia that presents shortly after birth and bone marrow granulocyte hypoplasia with maturation arrest at promyelocyte stage

A

Kostmann syndrome

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13
Q

gene that codes for neutrophil elastase

A

ELANE / ELA2

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14
Q

approximately 50% of px have mutations in ELANE/ELA2 and have periods of severe neutropenia every 21 days

A

cyclic neutropenia

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15
Q

shows more immature neutrophils than mature neutrophils, suggesting that cells are lost during maturation

A

chronic idiopathic neutropenia

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16
Q

autosomal recessive or x-linked inherited disease characterized by variable degrees of bone marrow failure, peripheral cytopenias, and increased risk for hematologic malignancies and other cancers

A

fanconi anemia

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17
Q

sex-linked recessive, autosomal dominant or autosomal recessively inherited disorder with a heterogenous presentation. patients have mucocutaneous abnormalities, abnormal skin pigmentation, nail dystrophy, and leukoplakia, bone marrow failure

A

dyskeratosis congenita

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18
Q

nonmalignant eosinophilia is generally caused by

A

cytokine stimulation especially from IL3 and IL5

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19
Q

major function of eosinophil wherein substances are released that damage and offending organism or target cell

A

degranulation

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20
Q

px with >1.5x10^9/L lasting more than 6 months without any identifiable cause, what is the diagnosis

A

hypereosinophilic syndrome

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21
Q

accompanied by neutrophilia during infection or inflammation

A

eosinophilia

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22
Q

common cause of basophilia

A

presence of malignant myeloproliferative neoplasm

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23
Q

frequently first sign of recovery from acute overwhelming infection or severe neutropenia (most common after cancer chemo) which is positive

A

monocytosis

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24
Q

absolute monocyte count of <0.2x10^9/L

A

monocytopenia

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25
found in patients receiving steroid therapy or hemodialysis or in sepsis. epstein-barr virus infected px
monocytopenia
26
reference range for relative lymphocytes
20-40%
27
an inner nuclear membrane protein that combines beta-type lamins and heterochromatin and plays a major role in leukocyte nuclear shape changes
lamin beta-receptor gene
28
autosomal dominant disorder characterized by decreased nuclear segmentation and coarse chromatin clumping pattern
pelger-huet anomaly
29
where is neutrophil hypersegmentation most often associated with?
megaloblastic anemia
30
it can be seen in myelodysplastic syndromes and represent a form of myeloid dysplasia.
neutrophil hypersegmentation
31
in this disorder, neutrophil hypersegmentation can be seen but px shows no sign of megaloblastic anemia
hereditary neutrophil hypersegmentation
32
hereditary condition characterized by normal granulocyte production but with impaired release into circulation that leads to neutropenia
myelokathexis
33
neutrophils appear hypermature, hypersegmented, hypercondensed chromatin, and pyknotic changes. cytoplasmic vacuoles may also be observed
myelokathexis
34
a syndrome in which warts, neutropenia, hypogammaglobinemia, infections, and myelokathexis are common findings
WHIM syndrome
35
incomplete degradation of mucopolysaccharides
alder-reilly anomaly
36
neutrophil that has a normal size 4-6 lobes in the nucleus found in the stage of recovery from infection
polycyte
37
Larger than normal neutrophil and has 5-10 nuclear lobes
macropolycyte
38
where is macropolycyte found?
pernicious anemia
39
Nucleus of the neutrophil becomes smaller and denser; nuclear segments disappear, leaving several balls of dense chromatin.
pyknocyte
40
Cell has a chromatin arrangement which gives the cell a “moth-eaten” or “tunneled” appearance or “swiss-cheese”. Cell has prominent azurophilic granules
virocyte/ atypical lymphocyte/ downey type cell/ turk irritation cell
41
It looks like a sunny side up egg. activated to respond to a viral infection. Or it can also respond to bacterial or parasitic infection.
virocyte/ atypical lymphocyte/ downey type cell/ turk irritation cell
42
Myeloblast that is characterized by having a nucleus with deep indentations often suggesting lobulations
rieder cell
43
where is rieder cell commonly seen in?
acute myeloid leukemia
44
Cell with holes or vacuoles in the cytoplasm. Signs of degeneration in severe infections, chemical poisoning and leukemia
vacuolated cell
45
Net-like nucleus from a ruptured white cell especially a PMN
basket cell/ smudge cell
46
where is basket cell/ smudge cell found?
chronic lymphocytic leukemia
47
the cause of this cell could be due to CLL or maybe the smear made is not good
basket cell/ smudge cell
48
A PMN which had engulfed the nuclear material of another PMN or a lymphocyte
lupus erythematous cell
49
There’s an ingestion or engulfment. Neutrophils or Macrophage that has phagocytized the denatured nuclear materials of other cells.
lupus erythematous cell
50
where is lupus erythematous cell found?
systemic lupus erythematous
51
A monocyte with an engulfed nucleus usually of a lymphocyte or maybe the whole lymphocyte itself
tart cell
52
what does tart cell exhibit?
necleophagocytosis
53
Lymphocytes with hair like cytoplasmic projections surrounding the nucleus
hairy cell
54
where is hairy cell found?
hairy cell leukemia
55
Rough lymph cell with nucleus that is grooved or convoluted
sezary cell
56
where is sezary cell found?
sezary syndrome, mycosis fungoides
57
Are linear or spindle-shaped red-purple inclusions in myeloblasts and monoblasts
auer bodies/ rods
58
derivatives of azurophilic granules
auer bodies/ rods
59
Caused by unusual development of lysosomes
auer bodies/ rods
60
cytoplasmic inclusion which result from abnormal fusion of primary azurophilic granules
auer bodies/ rods
61
what is the classification of auer rods?
pathological
62
These are red-staining needle-like bodies seen in the cytoplasm of either the myeloblast or monoblast.
auer bodies/ rods
63
Dark blue to purple cytoplasmic granules in the metamyelocyte, band or in neutrophil stage
toxic granules
64
Remnants of free ribosomes from an earlier stage of development
double-amato bodies
65
cytoplasmic inclusion mostly seen in bacterial infections, severe burns, exposure to cytotoxic agents and complicated pregnancies
double-amato bodies
66
Found in the cytoplasm of multiple myeloma and plasma cells after therapy with amidine drugs. Has an intracytoplasmic spherical shape
snapper-scheid bodies
67
Gamma globulins bodies in the cytoplasm of plasma cells and inflamed tissue
russell/ fuch's bodies
68
Bodies which gave a grape or berry or morula cell appearance
russell/ fuch's bodies
69
Has large peroxidase lysosomes inclusions that are deficient in enzymes for phagocytosis
Chediak-Higashi Syndrome
70
associated condition for chediak-higashi syndrome
albinism
71
Dohle bodies, thrombocytop enia,giant platelets and leukopenia
may-hegglin anomaly
72
vacuolization of leukocytes
Jordan's Anomaly
73
Peroxidase depletion in PMN and monocytes
Alius–Grignaschi Anomaly
74
Random movement of phagocytes is normal, but directional motility is impaired
job's syndrome
75
both random and directed movement of the cells are defective
lazy leukocyte syndrome
76
intracellular killing mechanism of granulocyte is defective. disease usually seen in childhood
Chronic Granulomatous Disease
77
phagocytes ingest but can’t kill catalase + organisms because of lack of appropriate respiratory burst
Chronic Granulomatous Disease
78
Asymptomatic carriers have half the normal C3 activity (heterozygous)
Congenital C₃ Deficiency
79
carriers fail to opsonize bacteria
Congenital C₃ Deficiency
80
MPO is decreased or absent in PMN and monocyte
Myeloperoxidase Deficiency
81
Deficiency of glucocerebrosidase enzyme
Gaucher’s Disease
82
deficiency in sphingomyelinase
Niemann-Pick Disease
83
macrophage with cholesterol overload due to increase in foam cells
Schuller-Christian Disease
84
This is the most common of the lysosomal lipid storage
Gaucher’s Disease
85
deficiency in hexosaminidase A
Tay-Sachs Disease
86
reduced Ig production in blood. associated with B-cell deficiency
Bruton Agammaglobulinemia
87
reduced production of Ig due to overactivity of T8 cells
common variable hypogamaglobulinemia
88
underdevelopment of the thymus; t-cell deficiency
Nezelof’s Syndrome
89
deletion of a small piece of chromosome 22
DiGeorge's syndrome
90
Loss of both T cell and B Cells function
Swiss-Type Agammaglobulinemia
91
Failure of T-cell response Only IgA and IgG are present; IgM is absent
Wiskott-Aldrich Syndrome
92
decreased T cell production; Characterized as having progressive loss of muscular coordination
ataxia telangiectasia
93
inherited leukocyte disorder caused by a mutation in the lamin B receptor
Pelger-Huet anomaly
94
inherited leukocyte disorder in which it is one of a group of disorders with mutations in nonmuscle myosin heavy-chain IIA?
May-Hegglin anomaly
95
What inherited leukocyte disorders might be seen in Hurler syndrome?
Alder-Reilly anomaly
96
lysosomal storage disease is characterized by macrophages with striated cytoplasm and storage of glucocerobroside
gaucher disease
97
the neutrophils in chronic granulomatous disease are incapable of producing:
hydrogen peroxide, hypochlorite, superoxide
98
individuals with X-linked SCID have a mutation that affects their ability to synthesize:
IL-2 receptor
99
an absolute lymphocytosis with reactive lymphocytes suggests what condition?
viral infection
100
what leukocyte cytoplasmic inclusion is composed of ribosomal RNA?
Dohle bodies

HEMA311 (5 decks)

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