HEMATOLOGY 311 nels

FINALS: LEUKOCYTE DISORDER

LEUKOCYTE DISORDER 4. RIEDER CELL

I. Morphological Abnormalities of Leukocytes

II. Non – Malignant Leukocyte Disorders
III. Malignant Leukocyte Disorders

MORPHOLOGICAL ABNORMALITIES OF LEUKOCYTE

1. HYPERSEGMENTED NEUTROPHIL
a. Polycyte
- Has a normal size but with 4 – 6 lobes in the • Myeloblast that is characterized by having a
nucleus. nucleus with deep indentions often suggesting
- Found in stage of recovery from infection. lobulations.
• Seen in:
b. Macropolycyte - Acute Myeloid Leukemia

5. VACUOLATED CELL

- Larger than normal neutrophil and has 5 – 10

nuclear lobes.
- Seen in Pernicious anemia and Megaloblastic
Anemia
• Cell with holes or vacuoles in the cytoplasm.
2. PYKNOCYTE • Signs of degeneration in severe infections,
• Nucleus becomes smaller and denser; nuclear chemical poisoning and leukemia.
segments disappear leaving several balls of dense
chromatin. 6. BASKET CELL/SMUDGE CELL

3. VIROCYTE OR ATYPICAL LYMPHOCYTE

• Net like – nucleus from ruptured white blood cell

especially Polymorpounclear (PMN)
• Atypical because they are larger and has more
-There is a fragility in the lymphocytes which tends
cytoplasm and has nucleoli in their nuclei.
to rupture when smear is made.
• Also called:
• Seen in:
- Downey Type Cell
- Chronic Lymphocytic Leukemia
- Turk Irritation Cell
• Cell has a chromatin arrangement which gives the
7. LUPUS ERYTHEMATOSUS CELL (LE CELL)
cell a “moth – eaten” or “tunneled” appearance
or “swiss – cheese”.
• Cell has prominent azurophilic granules.
• Seen in:
- Infectious mononucleosis
- Viral hepatitis
- Viral pneumonia
- Herpes simplex infections

Reference: Mrs. Agnes Guzman, RMT

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•A PMN which had engulfed the nuclear material of syndrome that is the most common form of
another PMN or a lymphocyte. cutaneous t-cell lymphoa that affects the skin.
• It has 2 nuclei: - LAP score → LR (Locomoid Rxn) → CML
a. Nucleus of Phagocyte
-Flattened in periphery ABNORMAL INCLUSION BODIES IN LEUKOCYTES
b. Ingested Nucleus 1. AUER BODIES/RODS
-Absent and replaced by a purplish homogenous -Are linear or spindle – shaped red purple inclusions
round mass. in myeloblasts and monoblasts.
*Spindle shape or needle-like in myeloblast and
8. TART CELL monoblast.
-Derivatives of azurophilic granules.
-Caused by unusual development of Lysosomes.

2. TOXIC GRANULES
-Dark blue to purple cytoplasmic granules in the
metamyelocyte, band or in neutrophil stage.
-Characteristics of bacterial infections and are frequently
seen in bacterial infection, Aplastic anemia and also in
myelosclerosis.
• A monocyte with an engulfed nucleus usually of a
lymphocyte or maybe the whole lymphocyte itself. 3. DOHLE – AMATO BODIES
• Exhibits nucleophagocytosis. -Small round or oval bodies up to 2 – 3um.
-Stain blue gray usually seen in the periphery of the
9. HAIRY CELL cytoplasm of neutrophils.
-Remnants of free ribosomes from an earlier stage of
development.
-Seen in:
✓ Bacterial Infections
✓ Severe Burns
✓ Exposure to Cytotoxic agents
✓ Complicated pregnancies
• Lymphocytes with hair like cytoplasmic projections
surrounding the nucleus. 4. SNAPPER – SCHEID BODIES
• Seen in: -Found in cytoplasm of multiple myeloma and plasma cells
- Hairy Cell Leukemia. after therapy with amidine drugs.
- HCL →Trap (Tartrate Resistance Acid
Phosphate) 5. RUSSELL OR FUCH’S BODIES
-Gamma globulins bodies in the cytoplasm of plasma
10. SEZARY CELL cells and inflamed tissue.
-Bodies which gave a grape or berry or morula cell
appearance.

TWO GROUPS OF LEUKOCYTE DISORDERS

A. Non – Neoplastic Disorders
̵ Not tumor related disorders
B. Neoplastic and Related Disorders
̵ Tumor related disorder
• Rough lymph cell with nucleus that is grooved or
convoluted.
• Hyperconvoluted cell
• Cancer of the skin
• Seen in:
- Sezary Syndrome a type of cutaneous t-cell
lymphoma.
- Mycosis fungoides also known as Alibert bazin
Reference: Mrs. Agnes Guzman, RMT
 HEMATOLOGY 311 nels
FINALS: LEUKOCYTE DISORDER
NON – MALIGNANT DISORDERS OF LEUKOCYTES

A. Granulocytes

DISORDER OF NUCLEUS DEFECT OR CHANGE ASSOCIATED CONDITIONS OTHERS

Hypersegmented Neutrophil Abnormal DNA Megaloblastic Anemia Shift to the right

synthesis -Autosomal dominant
-Undritz anomaly
Pelger – Huet Anomaly (PHA) Decreased Chronic Myeloid Leukemia -Hyposegmentation
segmentation in (CML) -Autosomal dominant
neutrophil “pinceness Myelodysplastic Syndrome *True PHA
appearance” (MDS) *Inherited

Dumbell shape or Decrease in lobulation

peanut shape because Eye glass
there is Dumbbell Shape
hyposegmentation. Peanut Shape
There is
hyposegmentation
because it lacks the
Lamin B which is
responsible for shape
change.

Mutation of Lamin B
PseudoPelger – Huet Anomaly Has less dense nuclei Burns Clinically significant
with hypogranular Drug reactions acquired phenomena
cytoplasm Infections
MDS
Associated with CML
malignant proliferative Acute Leukemia
disorder Chemotherapy

DISORDER OF DEFECT OR CHANGE ASSOCIATED CONDITIONS OTHERS

CYTOPLASM
Alder – Reily Anomaly Accumulation of degraded Hunter’s Syndrome (Type Autosomal recessive
mucopolysaccharides II)-enlarged head and brain Very short life
and heart damage. expenctancy
Incomplete degradation of
mucopolysaccharides. Hurler’s Syndrome
(Type I)-severe form of
Appearance of other reily mucopolysaccharidosis
body has this heavy toxic with skeletal abnormalities,
granulation. damage to brain and heart.

*Both has deficiency of

mucopolysaccharide.
Chediak – Higashi Has large peroxidase Albinism Fatal Autosomal recessive
Syndrome lysosomes inclusions that Recurrent infection Increase susceptibility to
are deficient in enzymes infection
for phagocytosis

Reference: Mrs. Agnes Guzman, RMT

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FINALS: LEUKOCYTE DISORDER
The problem is the lyst
gene.

May – Hegglin Anomaly Dohle bodies, Autosomal dominant

thrombocytopenia, giant
platelets and leucopenia.
Prolonged bleeding type
will also be present but
there is a normal platelet
aggregation.

Main problem is MYH9

gene.

Is caused by the mutation

of MYH9 which disrupts the
production of Myosin
Heavy Chain Type 2A gene
(MYH9) in which it affects
the megakaryocyte
maturation.

Mutation of MYH9 seen

also in:
Sebastian
Fechtner
Epstein Barr Syndrome
Jordan’s Anomaly Vacuolization of
leukocytes, monocytes and
lymphocytes.

Hepatosplenomegaly is
present.

Associated with neutral

lipid storage disease.

The gene involved has a

defective triglyceride
breakdown
Alius – Grignashi Anomaly Peroxidase depletion in Autosomal recessive
PMN and monocytes

Main problem is
Myeloperoxidase
deficiency.
*If myeloperoxidase
deficiency is involved, there
would be a recurrent
infection.

Reference: Mrs. Agnes Guzman, RMT

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FINALS: LEUKOCYTE DISORDER
FUNCTIONAL DISORDER DEFECT OR CHANGE ASSOCIATED CONDITIONS OTHERS

Job’s Syndrome Random movement of Severe recurrent infection Hyperimmunoglobulin E

phagocytes is normal, but
chemotactic and
directional motility is
impaired.
Lazy Leukocyte Syndrome Both random and
directional movement of
the cells are defective
Chronic Granulomatous Impaired NADPH oxidase
Disease (CGD)
Intracellular killing
mechanism of granulocyte
is defective
Persistent boils *Increase of total IgE
Cells respond slowly to
chemotactic factors
Recurrent mucous
membrane infections
Disease usually seen in Phagocytes ingest but can’t
childhood kill catalase + organisms
because of lack of
appropriate respiratory
burst
*To evaluate respiratory
burst we need to use Nitro-
blue tetrazolium test.

x - linked
Congenital C3 Deficiency Asymptomatic carriers Results in repeated Rare Autosomal recessive
have half the normal C3 infections trait
activity (heterozygous)

Homozygous carriers fail

to opsonize bacteria
*Therefore, there would be
a recurrent infection
Myeloperoxidase MPO is decreased or Without MPO, bacterial
Deficiency absent in PMN and killing is slowed
monocytes
*MPO is the granules
present in neutrophils.
Without this, bacterial
killing is slow.

B. Monocyte – Macrophage

DISORDER DEFECT OR CHANGE ASSOCIATED CONDITIONS OTHERS

Gaucher’s Disease Deficiency of Effects the bone marrow, Macrophages are with
glucocerebrosidase spleen and liver wrinkled – looking
enzyme cytoplasm and with small
*Glucocerebrosidase eccentric nucleus
enzyme are necessary for
the glycolipid metabolism. Decrease platelet and
anemia.
We have a triad use in the
diagnosis of Gaucher’s Treatment can be enzyme
disease. We have the: replacement.
*Hepatomegaly
*Gaucher’s cell in bone
marrow (in order to see the
Gaucher’s cells in bone

Reference: Mrs. Agnes Guzman, RMT

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FINALS: LEUKOCYTE DISORDER
marrow, we need to have
periodic Schiff stain)
*Increase serum
phosphatase

To confirm the presence of

Gaucher’s disease we need
to do gene testing.

Type I Non – neuropathic Adult type

Type II Acute neuropathic Infancy type
Type III Sub – acute neuropathic Childhood type

Niemann – Pick Disease Deficiency of Symptoms: Autosomal recessive

Sphingomyelinase *Enlarged spleen, liver Abnormal accumulation of
Type A *Intellectual decline sphingomyelin and
Acute Neuropathic Lipid storage disease. *Splurged speech cholesterol in body cells
manifested during infancy
(2 – 4 months) Main problem is No known treatment but
involves brain sphingomyelinase only the supportive
hepatosplenomegaly deficiency. treatment.

Type B
Chronic Form
without CN involvement

Type CD
Chronic Neuropathic

Type E
Adult
Non - Neuropathic
Schuller – Christian Macrophage with Hyperlipidemia
Disease cholesterol overload due
to increase in foam cells

C. Lymphocytes: disorders associated with t and b-cells.

DISORDER DEFECT OR CHANGE ASSOCIATED CONDITIONS OTHERS

Tay – Sachs Disease Deficiency of Autosomal recessive

hexosaminidase A Presence of Vacuolated
*Passed from parent to lymphocytes would be
child. This enzyme plays a seen
critical role in brain and
spinal cord. These enzymes
are found in the lysosome
and helps in the
breakdown of fatty
substances.
Bruton Reduced Ig production in B cell deficiency Inherited infantile sex –
Agammaglobulinemia blood linked
*Passed by the mother to Usually affects males
her male children
Reference: Mrs. Agnes Guzman, RMT
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FINALS: LEUKOCYTE DISORDER
All classes of
immunoglobulin are low.
Common Variable Reduced production of Ig B cell deficiency
Hypogammaglobulinemia due to overactivity of T8
cells
Nezelof’s Syndrome Underdevelopment of the T cell deficiency Autosomal recessive
thymus Congenital
immunodeficiency
Di George’s Syndrome Deletion of a small piece T cell deficiency Cause is unknown
of Chromosome 22
(Philadelphia
Chromosome)
Swiss – Type Loss of both T and B cells Severe combined Autosomal recessive
Agammaglobulinemia function immunodeficiency
disorders
Wiskott – Aldrich Failure of T cell response Severe combined Inherited as rare x – linked
Syndrome (WAS) Only IgA and IgG are immunodeficiency recessive
*Remember WATER. present; IgM is absent disorders
WA (Wiskott-Aldrich) Bleeding
Thrombocytopenia Thrombocytopenia
Eczema Recurrent Infection
Recurrent infection Eczema
Ataxia Telangiectasia Decreased T cell Rare childhood disease Characterized as having
*Ataxia: progressive loss of production Affects the brain and other progressive loss of
muscular coordination parts of the body muscular coordination
* Telangiectasia: dilation of Decrease IgA and IgE and
small blood vessels normal to increase IgG.

Reference: Mrs. Agnes Guzman, RMT