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OZAIR S.

AMBAG, RMT

WHITE BLOOD
CELL ANOMALIES
CONDITIONS/ ABNORMAL
WBCS OR INCLUSIONS

Smudge Cells
nuclear remnant of lymphocytes
appearance similar to a thumbprint
structureless chromatin
Associated conditions:
may be found in few numbers
may be associated with chronic
lymphocytic leukemia (CLL)

Basket Cells
nuclear remnants of granulocytic
cells
netlike chromatic pattern
associated conditions:
may be found in few numbers
may be seen in some leukemias
Leukocyte Adhesion Deficiency
lack of cell-surface adhesion proteins
affecting numerous cell functions.
failure to express cell surface
integrin (CD18) which is necessary
for leukocytes to adhere to the
vessel wall at the site of infection.
normal cell morphology
serious condition with recurrent
infections and high mortality

Myeloperoxidase Deficiency
defects in primary granules
most common neutrophil abnormality
low or absent myeloperoxidase
enzyme
normal cell morphology
commonly benign (other bactericidal
systems prevent most infections)
usually have mild symptoms only.
Chronic Granulomatous Disease (CGD)
an inherited disorder characterized
Nitroblue Tetrazolium
by defects in the respiratory burst
Test
oxidase system (cells engulf but are
unable to kill microorganisms)
normal neutrophil morphology
formation of granulomas (sites of
Abnormal Normal
chronic inflammation involving
primarily large macrophage) can
obstruct hollow organs
frequent infections especially in Chronic Granulomatous
children Disease (CGD)
TESTS:
Chemiluminescence Myeloperoxidase
Nitroblue Tetrazolium Test Deficiency

(NBT)
Nitroblue Tetrazolium Test (NBT)
semiquantitative assay
tests the phagocytic functions of
polymorphonuclear leukocytes
(PMNL)
PRINCIPLE:
increased enzyme activity
Purple to Blue Yellow normally present in neutrophil
during a bacterial infection is
capable of reducing colorless
nitroblue tetrazolium to blue-back
deposits of formazan.
In CGD, the neutrophils do not have
the normal ability to kill certain
organisms and are also unable to
reduce nitroblue tetrazolium.
Niemann-Pick Disease (NPD)
deficiency of sphingomyelinase (an
enzyme needed to break down
lipids)
FOAM CELL:
characteristic cells of NPD.
a.k.a Pick Cells
abnormal macrophage
whose cytoplasm is swollen
with small lipid droplets
presence of Zebra bodies
rare autosomal recessive disease
more commonly seen in Ashkenazi
Jews
signs of the disease begin in infancy
with poor physical development
spleen and liver are greatly
enlarged
disease is often fatal by three years
of age
Gaucher Disease
defect in the catabolic enzyme: -
glucocerebrosidase (an enzyme
that catalyzes the hydrolytic
cleavage of glucose from
glucocerebroside)
most common of the lipidoses
GAUCHER CELL
found in the bone marrow
large macrophage with
small, eccentric nucleus
cytoplasm is distended by
glucocerebrosides
(crumpled tissue paper)
Hypersegmented Neutrophil
nucleus has 5 to 10 lobes Undritz Anomaly
found in pernicious anemia, folic hyper segmentation of nuclei of
acid deficiency, Undritz anomaly, polymorphonuclear leukocytes.
myelokathexis.
Myelokathexis
is a congenital disorder of the
white blood cells that causes
severe, chronic leukopenia (a
reduction of circulating white
blood cells) and neutropenia (a
reduction of neutrophil
granulocytes).
the disorder is believed to be
inherited in an autosomal
dominant manner.
Myelokathexis refers to retention
(kathexis) of neutrophils in the
bone marrow (myelo).
Pelger-Huet Anomaly
nucleus has 1 to 2 lobes
hypo segmented neutrophils
most common genetic disorder of
WBCS
failure of the neutrophil nucleus to
segment
Pince-nez or spectacle form
of neutrophil nucleus
most apparent in neutrophils

Acquired/Pseudo - Pelger-Huet Anomaly


may be produce in the course of the following disorders:
Acute Myelogenous Leukemia
Chronic Myelogenous Leukemia
Myelodysplastic Syndrome
are a group of cancers in which immature blood
cells in the bone marrow do not mature and
become healthy blood cells.
Lupus Erythrematosus (LE) Cell
usually a neutrophil that has
ingested the antibody-coated
nucleus of another neutrophil or
has engulfed the homogenous,
globular nuclear mass of a
destroyed cell
usually an in vitro phenomenon
found in systemic lupus
erythrematosus (SLE) but may
also be found in comparable
connective tissue disorder

Reider Cell
similar to normal lymphocytes BUT
the nucleus is notched, lobulated
and cloverleaf-like
found in chronic lymphocytic
leukemia or can be artificially
formed through blood film
preparation.
Flame Cell
a.ka. Thesaurocyte
an abnormal plasma cell with
intensely eosinophilic or
flaming cytoplasm
found in IgA Myeloma

Grape Cell
a.ka. morula cell/mott cell
a abnormal plasma cell with a
cytoplasm that completely filled
with Russel Bodies
found in Multiple Myeloma
Alder-Reilly Anomaly
presence of Alder-Reilly bodies
characterized by dense azurophilic
granulation in all types of leukocytes
granulation results from an abnormal
deposition and storage of
mucopolysaccharides
found in mucopolysaccharidoses
Hunter Syndrome
Two neutrophils from a patient
Hurler Syndrome
with Alder-Reilly anomaly. Note
Sanfilippo Syndrome
the dark granules present in
both cells. Such granules may
also be seen in eosinophils and
basophils.
Chediak-Higashi Syndrome
is inherited as an autosomal recessive trait.
defective gene: LYST (Lysosome Trafficking Regulator)
defect in the lysosomes (cytoplasmic granules that are involved with the destruction of material
ingested by phagocytic cells).
characterized by the presence of large, abnormal cytoplasmic granules in phagocytes
(granulocytes and monocytes) and occasionally in lymphocytes
abnormal granules in phagocytes are: Peroxidase (+)
abnormal granules in lymphocytes are: Peroxidase (-)

partial albinism is observed


due to abnormal packaging
of melanosomes
patient has silver hair, pale
skin and suffers from
photophobia
Hairy Cells
small lymphocytes with little
cytoplasmic projections
Tartrate-Resistant Acid Phosphatase
(TRAP) - (+)
they are medium-sized cells with a
diameter of 15 to 30 um.
nucleus is round to oval in shape with
fine chromatin and may contain one to
five distinct nucleoli.
there is a small to moderate amount of
grayish cytoplasm that has hairlike
projections around the outer border of
a.k.a Leukemic Reticuloendotheliosis
cell. is a chronic, malignant
found only in Hairy Cell Leukemia
lymphoproliferative disorder.
nearly all blood cells contain 7 it is characterized by the presence of
nonerythroid isoenzymes of acid variable numbers of a distinct type of
phosphatase (0, 1, 2, 3, 3b, 4 and 5) cell called hairy cells in the blood
Isoenzyme 5 (tartrate resistant and is and bone marrow
produced in abundance by Hairy cells)
Tart Cells Toxic Granulations
a monocyte that has ingested a whole appears in neutrophils
lymphocyte or a nucleus (with an altered primary granules (present
identifiable nuclear chromatin) because of rapid cell maturation)
may be seen in drug sensitivity described as dark-blue to black
granules found in the cytoplasm of
neutrophils
seen in severe infections and chemical
poisoning
Auer Rods Reed-Sternberg Cells
a.k.a Faggot Cells a large lymphoid cell which may
bundles of sticks/ auer rods in the demonstrate two nuclei (with
cytoplasm eosinophilic nucleoli) and an
linear projections of primary granules abundant cytoplasm
seen in certain types of acute owls eye appearance
myelogenous leukemia (AML) presence of these cells in the definitive
histologic characteristics of Hodgkins
Disease
Lazy Leukocyte Syndrome
poor neutrophil response to
both syndrome is
chemotactic agents
deficient random mobility of characterized by
abnormal chemotactic
neutrophils
also characterized by neutropenia movement
Lazy Leukocyte Syndrome:
abnormal random
movement
Job Syndrome: normal
Job Syndrome random movement
a.k.a Hyperimmunoglobulinemia
neutrophils have poor directional mobility
also characterized by recurrent severe
bacterial infections, skeletal abnormalities
and elevated levels of IgE
TRIAD:
recurrent staphylococcal
associated with increased IgE
infection
serum levels, leading to the
pulmonary infection
name Autosomal Dominant
eczematous dermatitis
Hyper IgE Syndrome (AD-
HIES)
Jordans Anomaly
characterized by the presence of fat-
containing vacuoles in granulocytes
and monocytes
may be seen in muscular dystrophy
and ichthyosis

Muscular dystrophy (MD)


is a group of muscle diseases that

results in increasing weakening


and breakdown of skeletal
muscles over time.

Ichthyosis
is a family of genetic skin

disorders characterized by dry,


scaling skin that may be
thickened or very thin. The prefix
"ichthy" is taken from the Greek
root for the word fish.
Dhle Bodies
round or oval blue-staining
cytoplasmic inclusions found in
neutrophils (arranged in parallel rows
and consisting of ribosomal RNA)
found in severe burns, infections and
in pregnancy

May-Hegglin Anomaly
characterized by the presence of
gray-blue spindle shaped inclusions in
the cytoplasm of granulocytes and
monocytes
also characterized by leukopenia,
variable thrombocytopenia and giant
platelets
Difference between May-Hegglin Anomaly Inclusions and Dhle Bodies

PAS Reaction
INCLUSIONS Size Shape Content
(Periodic Acid Schiff)

made up of
MHA Inclusions Large Spindle-shaped Negative mRNA
(messenger
RNA)

made up of
Dhle Bodies Small Round Positive rRNA
(ribosomal
RNA)