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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
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 (-)
Ichthyosis
is a family of genetic skin
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)