MODULE 6 UNIT 2: WHITE BLOOD CELL ANOMALIES Flashcards
Owing to the fact that white blood cells are a heterogenous population, anomalies of white blood cells can be classified according to the
type of cell, cell parts affected and function
A. NUCLEAR ABNORMALITIES
- Hyposegmentation
- Hypersegmentation
Barr body (Sex chromatin)
B. CYTOPLASMIC ABNORMALITIES
- Alder-Reilly Bodies
- Auer Rods
- Chediak-Higashi granules
- Dohle Bodies
- Toxic granules
- Toxic vacuoles
C. ABNORMALITIES OF CELLS EXHIBITING PHAGOCYTOSIS
- LE cell
- Tart Cell
D. FUNCTION ABNORMALITIES
- Job syndrome
- Lazy Leukocyte Syndrome
- Chronic granulomatous disease
- Leukocyte Adhesion Disorder- I (LAD-I)
E. ABNORMALITIES ASSOCIATED WITH LYMPHOCYTES
- Atypical lymphocytes
- Basket cell/ Smudge cell
- Hairy cell
- Sezary cell
F. ABNORMALITIES ASSOCIATED WITH PLASMA CELLS
- Flame Cell
- Russell Bodies
- Grape Cell/ Berry Cell/ Morula Cell/ Mott Cell
- Dutcher’s Bodies
G. MONOCYTE/ MACROPHAGE LYSOSOMAL STORAGE DISORDERS
Lipid Storage Diseases
Mucopolysaccharidosis
Lipid Storage Diseases
Gaucher Disease
NiemannPick Disease
Fabry Disease
Tay-Sachs disease
Sandhoff disease
Sea Blue Histiocytes
Mucopolysaccharidosis
MPS I- Severe
MPS I- Attenuated
MPS II
MPS III
MPS IV
benign anomaly of neutrophils
Hyposegmentation
nucleus fails to segment properly
Hyposegmentation
bilobed nuclei
Hyposegmentation
o Dumbbell-shaped
o Spectacle-shaped
o Peanut-shaped
Hyposegmentation
o “Pince-nez”
Hyposegmentation
abnormality in the maturation of the neutrophils (abnormality in DNA synthesis)
Hypersegmentation
6 or more lobed nucleus
Hypersegmentation
o seen in the nuclear material
Appendage
o represents the second X chromosome in females o may be seen in 2-3% of neutrophils in females
Appendage
o NOT FOUND IN NORMAL MALES
Barr body
o a small, well-defined, round projection of nuclear chromatin
Barr body
o connected to the nucleus of the neutrophil by a single, fine strand of chromatin
Barr body
large purple-black coarse cytoplasmic granules
Alder-Reilly Bodies
accumulations of degraded mucopolysaccharides
Alder-Reilly Bodies
may be found in all leukocytes in cases of its diseases
Alder-Reilly Bodies
may resemble toxic granules
Alder-Reilly Bodies
used to differentiate Alder-Reilly Bodies
o Cetyl Trimethyl Ammonium Bromide (CTAB) Test
occurrence of white turbidity
Alder-Reilly Bodies
pink or red rod-shaped cytoplasmic structures
Auer Rods
formed from the fusion of primary granules
Auer Rods
normally found in the younger myeloid precursors
Auer Rods
When seen in mature cells, they are associated with AML or AMML
Auer Rods
giant red, blue to grayish round inclusions
Chediak-Higashi granules
deficient in enzymes for phagocytosis
Chediak-Higashi granules
giant lysosomal granules
o lymphocyte
o Neutrophil
o monocyte
Chediak-Higashi granules
peroxidase (+) Sudan Black B (+)
Chediak-Higashi granules
Amato Bodies
Dohle Bodies
single or multiple blue cytoplasmic inclusions in the neutrophil
Dohle Bodies
aggregates of free ribosomes or rough endoplasmic reticulum
Dohle Bodies
often confused with May-Hegglin anomaly
Dohle Bodies
leukocyte inclusions are composed of precipitated myosin heavy chains
Dohle Bodies
large purple to black azurophilic granules
Toxic granules
thought to be primary granules
Toxic granules
often present with Dohle bodies and toxic vacuoles
Toxic granules
↑ ALP activity
Toxic granules
large purple to black azurophilic granules
Toxic vacuoles
thought to be primary granules
Toxic vacuoles
↑ ALP activity
Toxic granules
often present with dohle bodies and toxic granules
. Toxic vacuoles
large empty white areas within cytoplasm
Toxic vacuoles
represent end-stage phagocytosis
Toxic vacuoles
neutrophil with large purple homogenous round inclusion with nucleus wrapped around
LE cell
appear smooth and evenly stained
LE cell
a monocyte with ingested lymphocyte
Tart Cell
appears rough & unevenly stained
Tart Cell
neutrophils exhibit normal random activity (chemokinesis) but abnormal directional activity (chemotaxis)
Job syndrome
Random locomotion
Chemokinesis
Directed locomotion brought about by CHEMOTAXINS (chemotactic factors such as endotoxins and other bacterial products, cytokines, and lymphokines)
Chemotaxis
Locomotion through unruptured walls of the blood vessels
Diapedesis
neutrophils have abnormal random and directional activity
Lazy Leukocyte Syndrome
inability of phagocytes to kill ingested microorganisms due to impaired NADPH oxidase/ Respiratory burst
Chronic granulomatous disease
diagnostic test: Nitroblue tetrazolium dye test
Chronic granulomatous disease
caused by decreased or truncated β2 integrin, needed for neutrophil adhesion to endothelial cells and recognition of bacteria
Leukocyte Adhesion Disorder- I (LAD-I)
caused by decreased amount or function of selectin ligands and defective leukocyte recruitment.
Leukocyte Adhesion DisorderII (LAD-II)
brought about by defective protein Kindlin-3, needed for β integrin activation and leukocyte rolling
Leukocyte Adhesion DisorderIII (LAD-III)
Failed response to external signals that would normally result in leukocyte activation
Leukocyte Adhesion DisorderIII (LAD-III)
reactive lymphocytes, variant lymphocytes, transformed lymphocytes, and leukocytoid lymphocytes
Atypical lymphocytes
can be referred to as DOWNEY CELLS (Classification by Dr. Hal Downey) of different types
Atypical lymphocytes
▪ Turk’s Irritation cells/ Plasmacytoid lymphocytes
Type I
▪ Characterized with a large block of chromatin
Type I
▪ IM cell
Type II
▪ Characterized by a round mass of chromatin; “BALLERINA SKIRT” appearance
Type II
▪ Seen in Infectious Mononucleosis
Type II Atypical lymphocytes
• Causative agent: Epstein-Barr virus
Infectious Mononucleosis
• Characterized by lymphocytosis often mistaken as monocytosis
Infectious Mononucleosis
T lymphocytes reacting to EBV-infected B lymphocytes
Atypical lymphocytes
▪ Vacoulated, “Swiss cheese” appearance, “Moth-eaten” appearance
Type III
white blood cells that have degenerated nucleus or ruptured cell
Basket cell/ Smudge cell
are B lymphocytes with hair like cytoplasmic projections surrounding the nucleus
Hairy cell
associated with Human T-lymphotropic virus Type II
Hairy cell
stain positive with tartrate resistant acid phosphatase (TRAP)
Hairy cell
a lymphocyte with T cell characteristics with nucleus
Sezary cell
grooved and have brain like convolutions (“cerebri” form)
Sezary cell
a plasma cell with red to pink cytoplasm associated with increase in immunoglobulin
Flame Cell
individual globules of immunoglobulins seen in plasma cells
Russell Bodies
are plasma cell that contains vacuoles and large protein globules
Grape Cell/ Berry Cell/ Morula Cell/ Mott Cell
occurs as the accumulation of Russell bodies
Grape Cell/ Berry Cell/ Morula Cell/ Mott Cell
intranuclear protein inclusions occurring in the plasma cell
Dutcher’s Bodies
Gaucher Disease
Deficient Enzyme
βglucocerebrosidase
Gaucher Disease
Accumulation of:
Glucocerebroside
Gaucher Disease
Morphologic Appearance
CYTOPLASM
Wrinkled/ Crumpled
“Cat-Scratch”
OTHERS
Clinical Triad used in Diagnosis
Hepatomegaly
Gaucher cells in BM
↑ serum phosphatase
NiemannPick Disease
Deficient Enzyme
Sphingomyelinase
NiemannPick Disease
Accumulation of:
Sphingomyelin
NiemannPick Disease
Morphologic Appearance
CYTOPLASM
Foamy
Fabry Disease
Deficient Enzyme
α-galactosidase
Fabry Disease
Accumulation of:
Ceramide trihexose
Fabry Disease
Morphologic Appearance
Tay-Sachs disease
Deficient Enzyme
Hexosaminidase A
Tay-Sachs disease
Accumulation of:
GM2 ganglioside
Tay-Sachs disease
Morphologic Appearance
CYTOPLASM
Vacuolated
Sandhoff disease
Deficient Enzyme
Hexosaminidase A
Hexosaminidase A & B
Sandhoff disease
Accumulation of:
Glycolipid & ganglioside
Sandhoff disease
Morphologic Appearance
CYTOPLASM
Vacuolated
Sea Blue Histiocytes
Deficient Enzyme
Unknown
Sea Blue Histiocytes
Accumulation of:
Unknown
Sea Blue Histiocytes
Morphologic Appearance
CYTOPLASM
Blue-green cytoplasm
MPS I- Severe
Deficient Enzyme: α-l-iduronidase
Accumulated substance:
Name:
Dermatan sulfate
Heparan sulfate
Hurler Syndrome
MPS I- Attenuated
Deficient Enzyme: α-l-iduronidase
Accumulated substance:
Name:
Dermatan sulfate
Heparan sulfate
Scheie Syndrome
MPS II
Deficient Enzyme: Iduronate sulfatase
Accumulated substance:
Name:
Dermatan sulfate
Heparan sulfate
Hunter Syndrome
MPS III
Deficient Enzyme:
Heparan-N-sulfate
aNacetylglucosamini dase
Acetyl–coenzyme A:aglucosaminide N-acetyltransferase
-
-
-Heparan sulfate
-Heparan sulfate
-Sanfilippo Syndrome Type A
-Sanfilippo Syndrome Type B
MPS IV
Deficient Enzyme: Galactose-6sulfatase
Accumulated substance:
Name:
Keratan sulfate
Chondroitin-6sulfate
Morquio syndrome Type A