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HEMA311 > Leukocyte Disorders > Flashcards

Leukocyte Disorders Flashcards

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
Q

found in patients receiving steroid therapy or hemodialysis or in sepsis. epstein-barr virus infected px

A

monocytopenia

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26
Q

reference range for relative lymphocytes

A

20-40%

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27
Q

an inner nuclear membrane protein that combines beta-type lamins and heterochromatin and plays a major role in leukocyte nuclear shape changes

A

lamin beta-receptor gene

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28
Q

autosomal dominant disorder characterized by decreased nuclear segmentation and coarse chromatin clumping pattern

A

pelger-huet anomaly

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29
Q

where is neutrophil hypersegmentation most often associated with?

A

megaloblastic anemia

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30
Q

it can be seen in myelodysplastic syndromes and represent a form of myeloid dysplasia.

A

neutrophil hypersegmentation

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31
Q

in this disorder, neutrophil hypersegmentation can be seen but px shows no sign of megaloblastic anemia

A

hereditary neutrophil hypersegmentation

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32
Q

hereditary condition characterized by normal granulocyte production but with impaired release into circulation that leads to neutropenia

A

myelokathexis

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33
Q

neutrophils appear hypermature, hypersegmented, hypercondensed chromatin, and pyknotic changes. cytoplasmic vacuoles may also be observed

A

myelokathexis

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34
Q

a syndrome in which warts, neutropenia, hypogammaglobinemia, infections, and myelokathexis are common findings

A

WHIM syndrome

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35
Q

incomplete degradation of mucopolysaccharides

A

alder-reilly anomaly

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36
Q

neutrophil that has a normal size 4-6 lobes in the nucleus found in the stage of recovery from infection

A

polycyte

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37
Q

Larger than normal neutrophil and has 5-10 nuclear lobes

A

macropolycyte

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38
Q

where is macropolycyte found?

A

pernicious anemia

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39
Q

Nucleus of the neutrophil becomes smaller and denser; nuclear segments disappear, leaving several balls of dense chromatin.

A

pyknocyte

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40
Q

Cell has a chromatin arrangement which
gives the cell a “moth-eaten” or “tunneled” appearance or “swiss-cheese”. Cell has prominent azurophilic granules

A

virocyte/ atypical lymphocyte/ downey type cell/ turk irritation cell

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41
Q

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.

A

virocyte/ atypical lymphocyte/ downey type cell/ turk irritation cell

42
Q

Myeloblast that is characterized by having a nucleus with deep indentations often suggesting lobulations

A

rieder cell

43
Q

where is rieder cell commonly seen in?

A

acute myeloid leukemia

44
Q

Cell with holes or vacuoles in the cytoplasm. Signs of degeneration in severe infections, chemical poisoning and leukemia

A

vacuolated cell

45
Q

Net-like nucleus from a ruptured white cell especially a PMN

A

basket cell/ smudge cell

46
Q

where is basket cell/ smudge cell found?

A

chronic lymphocytic leukemia

47
Q

the cause of this cell could be due to CLL or maybe the smear made is not good

A

basket cell/ smudge cell

48
Q

A PMN which had engulfed the nuclear material of another PMN or a lymphocyte

A

lupus erythematous cell

49
Q

There’s an ingestion or engulfment. Neutrophils or Macrophage that has phagocytized the denatured nuclear materials of other cells.

A

lupus erythematous cell

50
Q

where is lupus erythematous cell found?

A

systemic lupus erythematous

51
Q

A monocyte with an engulfed nucleus usually of a lymphocyte or maybe the whole lymphocyte itself

A

tart cell

52
Q

what does tart cell exhibit?

A

necleophagocytosis

53
Q

Lymphocytes with hair like cytoplasmic projections surrounding the nucleus

A

hairy cell

54
Q

where is hairy cell found?

A

hairy cell leukemia

55
Q

Rough lymph cell with nucleus that is grooved or convoluted

A

sezary cell

56
Q

where is sezary cell found?

A

sezary syndrome, mycosis fungoides

57
Q

Are linear or spindle-shaped red-purple inclusions in myeloblasts and monoblasts

A

auer bodies/ rods

58
Q

derivatives of azurophilic granules

A

auer bodies/ rods

59
Q

Caused by unusual development of lysosomes

A

auer bodies/ rods

60
Q

cytoplasmic inclusion which result from abnormal fusion of primary azurophilic granules

A

auer bodies/ rods

61
Q

what is the classification of auer rods?

A

pathological

62
Q

These are red-staining needle-like bodies seen in the cytoplasm of either the myeloblast or monoblast.

A

auer bodies/ rods

63
Q

Dark blue to purple cytoplasmic granules in the metamyelocyte, band or in neutrophil stage

A

toxic granules

64
Q

Remnants of free ribosomes from an earlier stage of development

A

double-amato bodies

65
Q

cytoplasmic inclusion mostly seen in bacterial infections, severe burns, exposure to cytotoxic agents and complicated pregnancies

A

double-amato bodies

66
Q

Found in the cytoplasm of multiple myeloma and plasma cells
after therapy with amidine drugs. Has an intracytoplasmic
spherical shape

A

snapper-scheid bodies

67
Q

Gamma globulins bodies in the cytoplasm of plasma cells and inflamed tissue

A

russell/ fuch’s bodies

68
Q

Bodies which gave a grape or berry or morula cell appearance

A

russell/ fuch’s bodies

69
Q

Has large peroxidase lysosomes inclusions that are deficient in enzymes for phagocytosis

A

Chediak-Higashi Syndrome

70
Q

associated condition for chediak-higashi syndrome

A

albinism

71
Q

Dohle bodies, thrombocytop enia,giant platelets and leukopenia

A

may-hegglin anomaly

72
Q

vacuolization of leukocytes

A

Jordan’s Anomaly

73
Q

Peroxidase depletion in PMN and monocytes

A

Alius–Grignaschi Anomaly

74
Q

Random movement of phagocytes is normal, but directional motility is impaired

A

job’s syndrome

75
Q

both random and directed movement of the cells are defective

A

lazy leukocyte syndrome

76
Q

intracellular killing mechanism of granulocyte is defective. disease usually seen in childhood

A

Chronic Granulomatous Disease

77
Q

phagocytes ingest but can’t kill catalase + organisms because of lack of appropriate respiratory burst

A

Chronic Granulomatous Disease

78
Q

Asymptomatic carriers have half the normal C3 activity (heterozygous)

A

Congenital C₃ Deficiency

79
Q

carriers fail to opsonize bacteria

A

Congenital C₃ Deficiency

80
Q

MPO is decreased or absent in PMN and monocyte

A

Myeloperoxidase Deficiency

81
Q

Deficiency of glucocerebrosidase enzyme

A

Gaucher’s Disease

82
Q

deficiency in sphingomyelinase

A

Niemann-Pick Disease

83
Q

macrophage with cholesterol overload due to increase in foam cells

A

Schuller-Christian Disease

84
Q

This is the most common of the lysosomal lipid storage

A

Gaucher’s Disease

85
Q

deficiency in hexosaminidase A

A

Tay-Sachs Disease

86
Q

reduced Ig production in blood. associated with B-cell deficiency

A

Bruton Agammaglobulinemia

87
Q

reduced production of Ig due to overactivity of T8 cells

A

common variable hypogamaglobulinemia

88
Q

underdevelopment of the thymus; t-cell deficiency

A

Nezelof’s Syndrome

89
Q

deletion of a small piece of chromosome 22

A

DiGeorge’s syndrome

90
Q

Loss of both T cell and B Cells function

A

Swiss-Type Agammaglobulinemia

91
Q

Failure of T-cell response Only IgA and IgG are present; IgM
is absent

A

Wiskott-Aldrich Syndrome

92
Q

decreased T cell production; Characterized as having progressive loss of muscular coordination

A

ataxia telangiectasia

93
Q

inherited leukocyte disorder caused by a mutation in the lamin B receptor

A

Pelger-Huet anomaly

94
Q

inherited leukocyte disorder in which it is one of a group of disorders with mutations in nonmuscle myosin heavy-chain IIA?

A

May-Hegglin anomaly

95
Q

What inherited leukocyte disorders might be seen in Hurler syndrome?

A

Alder-Reilly anomaly

96
Q

lysosomal storage disease is characterized by macrophages with striated cytoplasm and storage of glucocerobroside

A

gaucher disease

97
Q

the neutrophils in chronic granulomatous disease are incapable of producing:

A

hydrogen peroxide, hypochlorite, superoxide

98
Q

individuals with X-linked SCID have a mutation that affects their ability to synthesize:

A

IL-2 receptor

99
Q

an absolute lymphocytosis with reactive lymphocytes suggests what condition?

A

viral infection

100
Q

what leukocyte cytoplasmic inclusion is composed of ribosomal RNA?

A

Dohle bodies

HEMA311 (5 decks)

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