WBC anomalies Flashcards
NUCLEAR ABNORMALITIES
Hyposegmentation
Hypersegmentation
Barr Body (Sex chromatin)
CYTOPLASMIC ABNORMALITIES
Alder Reilly Bodies
Auer Bodies
Chediak-Higashi Granules
Dohle Bodies
Toxic Granules
Toxic Vacuoles
ABNORMALITIES OF CELLS EXHIBITING PHAGOCYTOSIS
LE cells
Tart cells
Functional Abnormalities
Job Syndrome
Lazy Leukocytes Syndrome
Leukocyte Adhesion Disorder I
LAD II
LAD III
ABNORMALITIES ASSOCIATED WITH LYMPHOCYTES
Atypical Lymphocytes
Basket Cell/ Smudge Cell
Hairy Cell
Sezary cell
ABNORMALITIES ASSOCIATED WITH PLASMA CELLS
Flame Cell
Russel bodies
Grape cell
Dutcher’s Bodies
MONOCYTE/MACROPHAGE LYSOSOMAL STORAGE DISORDERS
Lipid Storage Diseases
Mucopolysaccharidosis
Lipid Storage Diseases
Gaucher Ds
Niemman-Pick Ds
Fabry Ds
Tay-Sachs Ds
Sandhoff Ds
Sea Blue Histocytes
Mucopolysaccharidosis
MPS I SEVERE
MPS I ATTENUATED
MPS II SEVERE
MPS III
MPS IV
BA of neutrophils, bilobed nuclei, dumbbell shaped/pince-nez
DA: I: Pelger-Huet Anomaly (Autosomal Disorder)
A:Pseudo Pelger-Huet Anomaly (Myelofibrosis)
Hyposegmentation
Abnormality in the mat. of N, abn in DNA synthesis
6 or more lobed nucleus
DA: I Undrits Anomaly
A: Megaloblastic Anemia
Hypersegmentation
Appendage represents the second X
chromosome in females (may be seen in
2-3% of neutrophils in females)
* NOT FOUND IN NORMAL MALE
* Small, well-defined, round projection of
nuclear chromatin that is connected to
the nucleus of the neutrophil by a single,
fine strand of chromatin
Barr Body
Large purple-black coarse cytoplasmic
granules
Accumulations of degraded
mucopolysaccharides
May resemble toxic granules, Cetyl
Trimethyl Ammonium Bromide (CTAB):
used for differentiation → Turbidity
indicative of Alder Reilly bodies
DA:
* Alder-Reilly Anomaly (autosomal
recessive)
* Mucopolysaccharidoses
* Hurler’s syndrome
* Hunter’s syndrome
Alder Reilly Bodies
- Pink or red rod-shaped
- Formed from the fusion of primary
granules - Normally found in the younger myeloid
precursors → peroxidase positive
DA: - Acute myeloid leukemia (AML)
- Acute myelomonocytic leukemia
(AMML)
Auer Rods
- Giant red, blue to grayish round inclusions
- Deficient in enzymes for phagocytosis
- Seen in lymphocytes, neutrophil, and
monocyte - Peroxidase and Sudan Black B positive
DA: CH- Syndrome
Chediak-Higashi Granules
- Amato bodies
- Single or multiple blue cytoplasmic
inclusions in the neutrophil that are
aggregates of free ribosomes or RER - Often confused with May-Hegglin
anomaly (the leukocyte inclusions in MayHegglin anomaly are composed of
precipitated myosin chains)
DA: SI, TS,B
Dohle Bodies
- Large purple to black azurophilic granules
thought to be primary granules → shows
increased ALP activity - Often present with Dohle bodies and toxic
vacuoles
DA: I,TS,B,M,CP
Toxic Granules
- Large empty white areas within cytoplasm
that represents end-stage phagocytosis
DA: S,SI,TS
Toxic Vacuoles
- A neutrophil with large purple
homogenous round inclusions with nucleus
wrapped around - Appear smooth and evenly stained
DA: Lupus erythematous
LE cells
- A monocyte with ingested lymphocyte
- Appears rough and 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 Leukocytes Syndrome
Caused by decreased or truncated 𝜷2 integrin, needed for neutrophil adhesion to endothelial
cells and recognition of bacteria that leads to:
o Recurrent infections
o Neutrophilia
o Lymphadenopathy
o Splenomegaly
o Skin lesions
LEUKOCYTE ADHESION DISORDER-I (LAD-I)
Caused by decreased amount or function of selectin ligands and defective leukocyte
recruitment
* Clinical findings
o Physical growth retardation
o Coarse face and/or other physical deformities
o Neurological defects
o Recurring infections
o Absent blood group H antigen
LAD II
Caused by defective protein Kindlin-3, needed for 𝜷 integrin activation and leukocyte rolling
* Failed response to external signals that would normally result in leukocyte activation
* Clinical findings:
o Recurrent bacterial and fungal infections (less severe than LAD-I)
o Decreased platelet GPIIbβ3 leading to bleeding
LAD III
Reactive lymphocytes/ variant
lymphocytes/ transformed lymphocytes/
leukocytoid lymphocytes
* DOWNY CELLS (classification by Dr. Hal
Downey)
TYPE I,II,III
Atypical Lymphocytes
- Turk’s irritation cells/Plasmacytoid
lymphocytes - Characterized with a large block of
chromatin
T I
- IM cell
- Characterized by a round mass of
chromatin - BALLERINA SKIRT appearance
- Seen in Infectious Mononucleosis
- Causative agent: Epstein-Barr virus
- Characterized by lymphocytosis often
mistaken as monocytosis (hence the
name mononucleosis) - The atypical lymphocytes are T
lymphocytes reacting to EBC-infected B
lymphocytes
T II
- Vacuolated
- “Swiss cheese” appearance
- “Moth-eaten” appearance
T III
- White blood cells that have degenerated
nucleus or ruptured cell in form of smudge
or basket
DA: Chronic Lymphocytic Leukemia
Basket/Smudge Cell
- B lymphocytes with hair like cytoplasmic
projections surrounding the nucleus - Stain positive with tartrate resistant acid
phosphatase (TRAP)
DA: * Hairy cell leukemia (associated with
Human T-lymphotropic virus Type II)
Hairy Cell
- Lymphocytes with T cell characteristics
with nucleus that is grooved and have
brain-like convolutions (“cerebri” form)
DA: * Sezary syndrome (leukemic phase) - Mycosis fungoides (leukemic phase)
- Cutaneous T-cell lymphoma
SEZARY CELL
- Plasma cell with red to pink cytoplasm
associated with increase in
immunoglobulin (IgA)
DA: * Multiple myeloma
Flame Cell
Individual globules of immunoglobulins
seen in plasma cells
Russel Bodies
- Plasma cells that contains vacuoles and
large protein globules (appearing like
grapes)
DA: * Multiple myeloma - Reactive states
Grape/Berry/Morula/Mott Cell
- Intranuclear protein inclusions
DUTCHER’S BODIES
DE: 𝛽-glucocerebrosidase
Accumulation of: Glucocerebroside
MA: * Wrinkled/clumped cytoplasm
* “Cat-scratch” cytoplasm
Others: Clinical triad used in the diagnosis:
* Hepatomegaly
* Gaucher cells in BM
* Increased serum phosphate
GAUCHER DISEASE
DE:Sphingomyelinase
Ao:Sphingomyelin
MA:* Foamy cytoplasm
Niemann-Pick Ds
DE:a-galactosidase
Ao:Ceramide trihexose
Fabry DS
DE:Hexosaminidase A
Ao:GM2 ganglioside
MA:* Vacuolated cytoplasm
TAY-SACHS DISEASE
MA: Blue Green Cytoplasm
Sea Blue Histocytes
DE:Rodak:
Hexosaminidase A
Hexosaminidase A & B
Ao:Glycolipid
Ganglioside
MA:* Vacuolated cytoplasm
SANDHOFF DISEASE
Name:Hurler Syndrome
Ao:a-I-iduronidase
MA:* Dermatan sulfate
* Heparan sulfate
MPS-I SEVERE
Name:Scheie Syndrome
DE:a-I-iduronidase
AS:* Dermatan sulfate
* Heparan sulfate
MPS-I ATTENUATED
Name:Hunter’s syndrome
DE: Iduronate sulfatase
AS:* Dermatan sulfate
* Heparan sulfate
MPS-II SEVERE
MPS III
Name:?
DE:Heparan-N-sulfate
AS:* Heparan sulfate
Sanfilippo Syndrome
MPS III
Name:?
DE:a-N-acetylglucosaminidase
AS:* Heparan sulfate
SS TB
MPS III
Name:?
DE:Acetyl-coenzyme A
a-glucosaminide
N-acetyltransferase
AS:* Heparan sulfate
SS TC
MPS IV
Name:?
DE:Galactose-6-sulfatase
AS:* Keratan sulfate
* Chondroitin-6-sulfate
Morquio Syndrome Type A
MPS IV
Name:?
DE:𝛽-galactosidase
AS: Keratan sulfate
Morquio Syndrome Type B
