MODULE 6 UNIT 2: WHITE BLOOD CELL ANOMALIES Flashcards

1
Q

Owing to the fact that white blood cells are a heterogenous population, anomalies of white blood cells can be classified according to the

A

type of cell, cell parts affected and function

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2
Q

A. NUCLEAR ABNORMALITIES

A
  1. Hyposegmentation
  2. Hypersegmentation
    Barr body (Sex chromatin)
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3
Q

B. CYTOPLASMIC ABNORMALITIES

A
  1. Alder-Reilly Bodies
  2. Auer Rods
  3. Chediak-Higashi granules
  4. Dohle Bodies
  5. Toxic granules
  6. Toxic vacuoles
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4
Q

C. ABNORMALITIES OF CELLS EXHIBITING PHAGOCYTOSIS

A
  1. LE cell
  2. Tart Cell
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5
Q

D. FUNCTION ABNORMALITIES

A
  1. Job syndrome
  2. Lazy Leukocyte Syndrome
  3. Chronic granulomatous disease
  4. Leukocyte Adhesion Disorder- I (LAD-I)
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6
Q

E. ABNORMALITIES ASSOCIATED WITH LYMPHOCYTES

A
  1. Atypical lymphocytes
  2. Basket cell/ Smudge cell
  3. Hairy cell
  4. Sezary cell
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7
Q

F. ABNORMALITIES ASSOCIATED WITH PLASMA CELLS

A
  1. Flame Cell
  2. Russell Bodies
  3. Grape Cell/ Berry Cell/ Morula Cell/ Mott Cell
  4. Dutcher’s Bodies
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8
Q

G. MONOCYTE/ MACROPHAGE LYSOSOMAL STORAGE DISORDERS

A

Lipid Storage Diseases
Mucopolysaccharidosis

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9
Q

Lipid Storage Diseases

A

Gaucher Disease
NiemannPick Disease
Fabry Disease
Tay-Sachs disease
Sandhoff disease
Sea Blue Histiocytes

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10
Q

Mucopolysaccharidosis

A

MPS I- Severe
MPS I- Attenuated
MPS II
MPS III
MPS IV

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11
Q

 benign anomaly of neutrophils

A

Hyposegmentation

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12
Q

 nucleus fails to segment properly

A

Hyposegmentation

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13
Q

 bilobed nuclei

A

Hyposegmentation

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14
Q

o Dumbbell-shaped
o Spectacle-shaped
o Peanut-shaped

A

Hyposegmentation

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15
Q

o “Pince-nez”

A

Hyposegmentation

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16
Q

 abnormality in the maturation of the neutrophils (abnormality in DNA synthesis)

A

Hypersegmentation

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17
Q

 6 or more lobed nucleus

A

Hypersegmentation

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18
Q

o seen in the nuclear material

A

Appendage

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19
Q

o represents the second X chromosome in females o may be seen in 2-3% of neutrophils in females

A

Appendage

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20
Q

o NOT FOUND IN NORMAL MALES

A

Barr body

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21
Q

o a small, well-defined, round projection of nuclear chromatin

A

Barr body

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22
Q

o connected to the nucleus of the neutrophil by a single, fine strand of chromatin

A

Barr body

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23
Q

 large purple-black coarse cytoplasmic granules

A

Alder-Reilly Bodies

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24
Q

 accumulations of degraded mucopolysaccharides

A

Alder-Reilly Bodies

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25
Q

 may be found in all leukocytes in cases of its diseases

A

Alder-Reilly Bodies

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26
Q

 may resemble toxic granules

A

Alder-Reilly Bodies

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27
Q

used to differentiate Alder-Reilly Bodies

A

o Cetyl Trimethyl Ammonium Bromide (CTAB) Test

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28
Q

occurrence of white turbidity

A

Alder-Reilly Bodies

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29
Q

 pink or red rod-shaped cytoplasmic structures

A

Auer Rods

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30
Q

 formed from the fusion of primary granules

A

Auer Rods

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31
Q

 normally found in the younger myeloid precursors

A

Auer Rods

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32
Q

 When seen in mature cells, they are associated with AML or AMML

A

Auer Rods

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33
Q

 giant red, blue to grayish round inclusions

A

Chediak-Higashi granules

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34
Q

 deficient in enzymes for phagocytosis

A

Chediak-Higashi granules

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35
Q

 giant lysosomal granules
o lymphocyte
o Neutrophil
o monocyte

A

Chediak-Higashi granules

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36
Q

 peroxidase (+)  Sudan Black B (+)

A

Chediak-Higashi granules

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37
Q

 Amato Bodies

A

Dohle Bodies

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38
Q

 single or multiple blue cytoplasmic inclusions in the neutrophil

A

Dohle Bodies

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39
Q

 aggregates of free ribosomes or rough endoplasmic reticulum

A

Dohle Bodies

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40
Q

 often confused with May-Hegglin anomaly

A

Dohle Bodies

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41
Q

leukocyte inclusions are composed of precipitated myosin heavy chains

A

Dohle Bodies

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42
Q

 large purple to black azurophilic granules

A

Toxic granules

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43
Q

 thought to be primary granules

A

Toxic granules

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44
Q

 often present with Dohle bodies and toxic vacuoles

A

Toxic granules

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45
Q

 ↑ ALP activity

A

Toxic granules

46
Q

 large purple to black azurophilic granules

A

Toxic vacuoles

47
Q

 thought to be primary granules

A

Toxic vacuoles

48
Q

 ↑ ALP activity

A

Toxic granules

49
Q

 often present with dohle bodies and toxic granules

A

. Toxic vacuoles

50
Q

 large empty white areas within cytoplasm

A

Toxic vacuoles

51
Q

 represent end-stage phagocytosis

A

Toxic vacuoles

52
Q

 neutrophil with large purple homogenous round inclusion with nucleus wrapped around

A

LE cell

53
Q

 appear smooth and evenly stained

A

LE cell

54
Q

 a monocyte with ingested lymphocyte

A

Tart Cell

55
Q

 appears rough & unevenly stained

A

Tart Cell

56
Q

 neutrophils exhibit normal random activity (chemokinesis) but abnormal directional activity (chemotaxis)

A

Job syndrome

57
Q

Random locomotion

A

Chemokinesis

58
Q

Directed locomotion brought about by CHEMOTAXINS (chemotactic factors such as endotoxins and other bacterial products, cytokines, and lymphokines)

A

Chemotaxis

59
Q

Locomotion through unruptured walls of the blood vessels

A

Diapedesis

60
Q

 neutrophils have abnormal random and directional activity

A

Lazy Leukocyte Syndrome

61
Q

 inability of phagocytes to kill ingested microorganisms due to impaired NADPH oxidase/ Respiratory burst

A

Chronic granulomatous disease

62
Q

 diagnostic test: Nitroblue tetrazolium dye test

A

Chronic granulomatous disease

63
Q

 caused by decreased or truncated β2 integrin, needed for neutrophil adhesion to endothelial cells and recognition of bacteria

A

Leukocyte Adhesion Disorder- I (LAD-I)

64
Q

 caused by decreased amount or function of selectin ligands and defective leukocyte recruitment.

A

Leukocyte Adhesion DisorderII (LAD-II)

65
Q

 brought about by defective protein Kindlin-3, needed for β integrin activation and leukocyte rolling

A

Leukocyte Adhesion DisorderIII (LAD-III)

66
Q

 Failed response to external signals that would normally result in leukocyte activation

A

Leukocyte Adhesion DisorderIII (LAD-III)

67
Q

 reactive lymphocytes, variant lymphocytes, transformed lymphocytes, and leukocytoid lymphocytes

A

Atypical lymphocytes

68
Q

 can be referred to as DOWNEY CELLS (Classification by Dr. Hal Downey) of different types

A

Atypical lymphocytes

69
Q

▪ Turk’s Irritation cells/ Plasmacytoid lymphocytes

A

Type I

70
Q

▪ Characterized with a large block of chromatin

A

Type I

71
Q

▪ IM cell

A

Type II

72
Q

▪ Characterized by a round mass of chromatin; “BALLERINA SKIRT” appearance

A

Type II

73
Q

▪ Seen in Infectious Mononucleosis

A

Type II Atypical lymphocytes

74
Q

• Causative agent: Epstein-Barr virus

A

Infectious Mononucleosis

75
Q

• Characterized by lymphocytosis often mistaken as monocytosis

A

Infectious Mononucleosis

76
Q

T lymphocytes reacting to EBV-infected B lymphocytes

A

Atypical lymphocytes

77
Q

▪ Vacoulated, “Swiss cheese” appearance, “Moth-eaten” appearance

A

Type III

78
Q

 white blood cells that have degenerated nucleus or ruptured cell

A

Basket cell/ Smudge cell

79
Q

 are B lymphocytes with hair like cytoplasmic projections surrounding the nucleus

A

Hairy cell

80
Q

associated with Human T-lymphotropic virus Type II

A

Hairy cell

81
Q

 stain positive with tartrate resistant acid phosphatase (TRAP)

A

Hairy cell

82
Q

 a lymphocyte with T cell characteristics with nucleus

A

Sezary cell

83
Q

 grooved and have brain like convolutions (“cerebri” form)

A

Sezary cell

84
Q

 a plasma cell with red to pink cytoplasm associated with increase in immunoglobulin

A

Flame Cell

85
Q

 individual globules of immunoglobulins seen in plasma cells

A

Russell Bodies

86
Q

 are plasma cell that contains vacuoles and large protein globules

A

Grape Cell/ Berry Cell/ Morula Cell/ Mott Cell

87
Q

 occurs as the accumulation of Russell bodies

A

Grape Cell/ Berry Cell/ Morula Cell/ Mott Cell

88
Q

 intranuclear protein inclusions occurring in the plasma cell

A

Dutcher’s Bodies

89
Q

Gaucher Disease

Deficient Enzyme

A

βglucocerebrosidase

90
Q

Gaucher Disease

Accumulation of:

A

Glucocerebroside

91
Q

Gaucher Disease

Morphologic Appearance

A

CYTOPLASM
 Wrinkled/ Crumpled
 “Cat-Scratch”

OTHERS
 Clinical Triad used in Diagnosis
 Hepatomegaly
 Gaucher cells in BM
 ↑ serum phosphatase

92
Q

NiemannPick Disease

Deficient Enzyme

A

Sphingomyelinase

93
Q

NiemannPick Disease

Accumulation of:

A

Sphingomyelin

94
Q

NiemannPick Disease

Morphologic Appearance

A

CYTOPLASM
 Foamy

95
Q

Fabry Disease

Deficient Enzyme

A

α-galactosidase

96
Q

Fabry Disease

Accumulation of:

A

Ceramide trihexose

97
Q

Fabry Disease

Morphologic Appearance

A
98
Q

Tay-Sachs disease

Deficient Enzyme

A

Hexosaminidase A

99
Q

Tay-Sachs disease

Accumulation of:

A

GM2 ganglioside

100
Q

Tay-Sachs disease

Morphologic Appearance

A

CYTOPLASM

 Vacuolated

101
Q

Sandhoff disease

Deficient Enzyme

A

Hexosaminidase A
Hexosaminidase A & B

102
Q

Sandhoff disease

Accumulation of:

A

Glycolipid & ganglioside

103
Q

Sandhoff disease

Morphologic Appearance

A

CYTOPLASM

 Vacuolated

104
Q

Sea Blue Histiocytes

Deficient Enzyme

A

Unknown

105
Q

Sea Blue Histiocytes

Accumulation of:

A

Unknown

106
Q

Sea Blue Histiocytes

Morphologic Appearance

A

CYTOPLASM

 Blue-green cytoplasm

107
Q

MPS I- Severe

Deficient Enzyme: α-l-iduronidase
Accumulated substance:
Name:

A

Dermatan sulfate
Heparan sulfate

Hurler Syndrome

108
Q

MPS I- Attenuated

Deficient Enzyme: α-l-iduronidase
Accumulated substance:
Name:

A

Dermatan sulfate
Heparan sulfate

Scheie Syndrome

109
Q

MPS II

Deficient Enzyme: Iduronate sulfatase
Accumulated substance:
Name:

A

Dermatan sulfate
Heparan sulfate

Hunter Syndrome

110
Q

MPS III

Deficient Enzyme:
Heparan-N-sulfate
aNacetylglucosamini dase
Acetyl–coenzyme A:aglucosaminide N-acetyltransferase

-

-

A

-Heparan sulfate
-Heparan sulfate

-Sanfilippo Syndrome Type A
-Sanfilippo Syndrome Type B

111
Q

MPS IV

Deficient Enzyme: Galactose-6sulfatase
Accumulated substance:
Name:

A

Keratan sulfate
Chondroitin-6sulfate

Morquio syndrome Type A