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
 may be found in all leukocytes in cases of its diseases
Alder-Reilly Bodies
26
 may resemble toxic granules
Alder-Reilly Bodies
27
used to differentiate Alder-Reilly Bodies
o Cetyl Trimethyl Ammonium Bromide (CTAB) Test
28
occurrence of white turbidity
Alder-Reilly Bodies
29
 pink or red rod-shaped cytoplasmic structures
Auer Rods
30
 formed from the fusion of primary granules
Auer Rods
31
 normally found in the younger myeloid precursors
Auer Rods
32
 When seen in mature cells, they are associated with AML or AMML
Auer Rods
33
 giant red, blue to grayish round inclusions
Chediak-Higashi granules
34
 deficient in enzymes for phagocytosis
Chediak-Higashi granules
35
 giant lysosomal granules o lymphocyte o Neutrophil o monocyte
Chediak-Higashi granules
36
 peroxidase (+)  Sudan Black B (+)
Chediak-Higashi granules
37
 Amato Bodies
Dohle Bodies
38
 single or multiple blue cytoplasmic inclusions in the neutrophil
Dohle Bodies
39
 aggregates of free ribosomes or rough endoplasmic reticulum
Dohle Bodies
40
 often confused with May-Hegglin anomaly
Dohle Bodies
41
leukocyte inclusions are composed of precipitated myosin heavy chains
Dohle Bodies
42
 large purple to black azurophilic granules
Toxic granules
43
 thought to be primary granules
Toxic granules
44
 often present with Dohle bodies and toxic vacuoles
Toxic granules
45
 ↑ ALP activity
Toxic granules
46
 large purple to black azurophilic granules
Toxic vacuoles
47
 thought to be primary granules
Toxic vacuoles
48
 ↑ ALP activity
Toxic granules
49
 often present with dohle bodies and toxic granules
. Toxic vacuoles
50
 large empty white areas within cytoplasm
Toxic vacuoles
51
 represent end-stage phagocytosis
Toxic vacuoles
52
 neutrophil with large purple homogenous round inclusion with nucleus wrapped around
LE cell
53
 appear smooth and evenly stained
LE cell
54
 a monocyte with ingested lymphocyte
Tart Cell
55
 appears rough & unevenly stained
Tart Cell
56
 neutrophils exhibit normal random activity (chemokinesis) but abnormal directional activity (chemotaxis)
Job syndrome
57
Random locomotion
Chemokinesis
58
Directed locomotion brought about by CHEMOTAXINS (chemotactic factors such as endotoxins and other bacterial products, cytokines, and lymphokines)
Chemotaxis
59
Locomotion through unruptured walls of the blood vessels
Diapedesis
60
 neutrophils have abnormal random and directional activity
Lazy Leukocyte Syndrome
61
 inability of phagocytes to kill ingested microorganisms due to impaired NADPH oxidase/ Respiratory burst
Chronic granulomatous disease
62
 diagnostic test: Nitroblue tetrazolium dye test
Chronic granulomatous disease
63
 caused by decreased or truncated β2 integrin, needed for neutrophil adhesion to endothelial cells and recognition of bacteria
Leukocyte Adhesion Disorder- I (LAD-I)
64
 caused by decreased amount or function of selectin ligands and defective leukocyte recruitment.
Leukocyte Adhesion DisorderII (LAD-II)
65
 brought about by defective protein Kindlin-3, needed for β integrin activation and leukocyte rolling
Leukocyte Adhesion DisorderIII (LAD-III)
66
 Failed response to external signals that would normally result in leukocyte activation
Leukocyte Adhesion DisorderIII (LAD-III)
67
 reactive lymphocytes, variant lymphocytes, transformed lymphocytes, and leukocytoid lymphocytes
Atypical lymphocytes
68
 can be referred to as DOWNEY CELLS (Classification by Dr. Hal Downey) of different types
Atypical lymphocytes
69
▪ Turk’s Irritation cells/ Plasmacytoid lymphocytes
Type I
70
▪ Characterized with a large block of chromatin
Type I
71
▪ IM cell
Type II
72
▪ Characterized by a round mass of chromatin; “BALLERINA SKIRT” appearance
Type II
73
▪ Seen in Infectious Mononucleosis
Type II Atypical lymphocytes
74
• Causative agent: Epstein-Barr virus
Infectious Mononucleosis
75
• Characterized by lymphocytosis often mistaken as monocytosis
Infectious Mononucleosis
76
T lymphocytes reacting to EBV-infected B lymphocytes
Atypical lymphocytes
77
▪ Vacoulated, “Swiss cheese” appearance, “Moth-eaten” appearance
Type III
78
 white blood cells that have degenerated nucleus or ruptured cell
Basket cell/ Smudge cell
79
 are B lymphocytes with hair like cytoplasmic projections surrounding the nucleus
Hairy cell
80
associated with Human T-lymphotropic virus Type II
Hairy cell
81
 stain positive with tartrate resistant acid phosphatase (TRAP)
Hairy cell
82
 a lymphocyte with T cell characteristics with nucleus
Sezary cell
83
 grooved and have brain like convolutions (“cerebri” form)
Sezary cell
84
 a plasma cell with red to pink cytoplasm associated with increase in immunoglobulin
Flame Cell
85
 individual globules of immunoglobulins seen in plasma cells
Russell Bodies
86
 are plasma cell that contains vacuoles and large protein globules
Grape Cell/ Berry Cell/ Morula Cell/ Mott Cell
87
 occurs as the accumulation of Russell bodies
Grape Cell/ Berry Cell/ Morula Cell/ Mott Cell
88
 intranuclear protein inclusions occurring in the plasma cell
Dutcher’s Bodies
89
Gaucher Disease Deficient Enzyme
βglucocerebrosidase
90
Gaucher Disease Accumulation of:
Glucocerebroside
91
Gaucher Disease Morphologic Appearance
CYTOPLASM  Wrinkled/ Crumpled  “Cat-Scratch” OTHERS  Clinical Triad used in Diagnosis  Hepatomegaly  Gaucher cells in BM  ↑ serum phosphatase
92
NiemannPick Disease Deficient Enzyme
Sphingomyelinase
93
NiemannPick Disease Accumulation of:
Sphingomyelin
94
NiemannPick Disease Morphologic Appearance
CYTOPLASM  Foamy
95
Fabry Disease Deficient Enzyme
α-galactosidase
96
Fabry Disease Accumulation of:
Ceramide trihexose
97
Fabry Disease Morphologic Appearance
---
98
Tay-Sachs disease Deficient Enzyme
Hexosaminidase A
99
Tay-Sachs disease Accumulation of:
GM2 ganglioside
100
Tay-Sachs disease Morphologic Appearance
CYTOPLASM  Vacuolated
101
Sandhoff disease Deficient Enzyme
Hexosaminidase A Hexosaminidase A & B
102
Sandhoff disease Accumulation of:
Glycolipid & ganglioside
103
Sandhoff disease Morphologic Appearance
CYTOPLASM  Vacuolated
104
Sea Blue Histiocytes Deficient Enzyme
Unknown
105
Sea Blue Histiocytes Accumulation of:
Unknown
106
Sea Blue Histiocytes Morphologic Appearance
CYTOPLASM  Blue-green cytoplasm
107
MPS I- Severe Deficient Enzyme: α-l-iduronidase Accumulated substance: Name:
Dermatan sulfate Heparan sulfate Hurler Syndrome
108
MPS I- Attenuated Deficient Enzyme: α-l-iduronidase Accumulated substance: Name:
Dermatan sulfate Heparan sulfate Scheie Syndrome
109
MPS II Deficient Enzyme: Iduronate sulfatase Accumulated substance: Name:
Dermatan sulfate Heparan sulfate Hunter Syndrome
110
MPS III Deficient Enzyme: Heparan-N-sulfate aNacetylglucosamini dase Acetyl–coenzyme A:aglucosaminide N-acetyltransferase Accumulated substance: - - Name: - -
-Heparan sulfate -Heparan sulfate -Sanfilippo Syndrome Type A -Sanfilippo Syndrome Type B
111
MPS IV Deficient Enzyme: Galactose-6sulfatase Accumulated substance: Name:
Keratan sulfate Chondroitin-6sulfate Morquio syndrome Type A