Ejs Leukocyte Non Malignant Abnormalities

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CLINICAL

HEMATOLOGY
JADE S. SUNICO, MSMT; JOHN MARK CAPUYAN, RMT
LEUKOCYTE
NON-MALIGNANT ABNORMALITIES
(QUANTITATIVE)
QUANTITATIVE LEUKOCYTE ABNORMALITIES

LEUKOCYTOSIS LEUKOPENIA

Neutrophilia Neutropenia
Eosinophilia Eosinopenia
Basophilia Basopenia
Monocytosis Monocytopenia
Lymphocytosis Lymphocytopenia
Neutrophil Quantitative Disorders

• NEUTROPHILIA • NEUTROPENIA
• Neutrophil count greater than • Most common cause of
7.0 – 8.0 x 109/L leukopenia
• Neutrophil count less than 1.75 to
• Causes: 1.8 x 109/L
➢ Infection • Causes:
➢ Inflammation ➢ Inherited – due to defective stem
➢ malignancy cell development
➢ Acquired – due to radiation,
chemicals (benzene) and drugs
(chloramphenicol)
Eosinophil Quantitative Disorders

• EOSINOPHILIA • EOSINOPENIA
• Eosinophil count greater than • Difficult to detect in routine
0.7 x 109/L differential count
• Maybe inherited or reactive • Zero is still considered normal
• Parasitic • Causes:
➢ Tissue hypersensitivity ➢ Acute infections
reactions (Skin diseases) ➢ BM Aplasia
➢ Transplant rejection
➢ MPD’s
➢ Asthma
Basophil Quantitative Disorders

• BASOPHILIA • BASOPENIA
• Basophil count greater than 0.3 • Difficult to detect in routine
x 109/L differential count
• Causes:
➢ Immediate Hypersensitivity, • Causes:
➢ MPD’s ➢ Stress,
➢ Ulcerative colitis ➢ Hyperthyroidism and
➢ Chronic inflammatory ➢ Steroid treatment
conditions
Monocyte Quantitative Disorders

• MONOCYTOSIS • MONOCYTOPENIA
• Monocyte count greater • Monocyte count less than
than 0.9 x 109/L 0.3 x 109/L
• Since mono and neutro share a
common stem cell, neutrophilia
Causes:
is also associated with
monocytosis ➢ administration of
• Causes: tuberculosis, SBE, glucocorticosteroid
syphilis, surgical trauma,
tumors, GI disease
tetrachloroethane poisoning
Lymphocyte Quantitative Disorders

• LYMPHOCYTOSIS • LYMPHOCYTOPENIA
• Lymphocyte count more than • Lymphocyte count less than 1.0 x
• 4.0 x 109/L (adult) or 109/L
• 9.0 x 109/L (infant) • Causes:
• Causes: ➢ HIV (CD4)
➢ EBV ➢ Malnutrition
➢ CMV ➢ Chemotherapy
➢ HIV (CD8) ➢ Radiation
➢ Hepatitis viruses ➢ Renal Failure
➢ Autoimmune disorders
Variant Lymphocyte Morphology
Also Virucytes;
described by Downey and McKinley
✓ Type I - Turk’s irritation cell; dense with round nuclei
✓ Type II- IM cell
✓ Type III- Transformed or reticular cell

Infectious mononucleosis,
CMV Cell showing a large activated
IM Cells
lymphoid cell in the blood
Variant Lymphocyte Morphology
• Reactive or Variant Lymphocyte Morphology
• Absolute Lymphocytosis with Variant Lymph Morphology (IM, CMV)
• Absolute Lymphocytosis with Normal Lymph Morphology (Bordetella)
• Relative Lymphocytosis with Variant Lymph Morphology (Toxoplasmosis)
• Relative Lymphocytosis with Normal Lymph Morphology (Neutropenia)
LEUKOCYTE
NON-MALIGNANT ABNORMALITIES
(QUALITATIVE)
QUALITIATIVE PHAGOCYTE DISORDERS

CYTOPLASMIC NUCLEAR
CHANGES CHANGES

• Toxic granulation • Hypersegmentation


• Toxic vacuolation • Hyposegmentation
• Dohle Amato bodies
Toxic Granulation

• Segmented neutrophils with


a dustlike appearance

• With prominent purple to


blue-black granules in the
cytoplasm of neutrophils
• Often associated with severe
infections
Toxic Vacuolation

• Cytoplasm of neutrophils contain


vacuoles (spaces).

• Vacuolization appears due to:


✓ prolonged exposure of blood to
drugs such as sulfonamide or
chloroquine
✓ prolonged storage leading to
phagocytosis or
autophagocytosis of granules
and cytoplasmic contents
Döhle Amato Bodies

• Cytoplasmic inclusions consist of ribosomal


RNA

• Rod-shaped, pale bluish-gray structure near


the cytoplasmic membrane

• Seen in burns, toxemia of pregnancy and


numerous infectious diseases (scarlet fever)

• Named after a German Pathologist,


• Dr. Karl Gottfried Paul Dohle (1855-1928) 1. Toxic granulation; 2. Dohle Bodies
Nuclear Changes
HYPERSEGMENTATION HYPOSEGMENTATION

• Neutrophil with 5 or more lobes


• Commonly seen in long-term • Failure of normal lobulation of
chronic infections neutrophil
• May either large or normal sized
HEREDITARY LEUKOCYTE DISORDERS

DEFECTIVE DEFECTIVE
MORPHOLOGY FUNCTION

1. May-Hegglin Anomaly 1. Job Syndrome


2. Alder’s (Alder-Reilly) Anomaly 2. Lazy Leukocyte Syndrome
3. Pelger-Huet Anomaly 3. Congenital C3 deficiency
4. Chediak-Higashi Syndrome 4. Chronic Granulomatous Disease
5. G6PD Deficiency
6. Myeloperoxidase Deficiency
May-Hegglin Anomaly

• Inherited disorder characterized by


thrombocytopenia and giant platelets

• Gray blue spindle-shaped inclusions


resembling Dohle Bodies in the
cytoplasm of granulocytes and
monocytes; not necessarily near the
cytoplasmic membrane

• This thought to be mRNA.

• First described by Dr. May in 1909 and


Dr. Hegglin in 1945 from Switzerland.
Alder-Reilly Anomaly

• Rare genetic disorder (autosomal


recessive) that is associated with
the presence of coarse, dark
granules in neutrophils,
lymphocytes, monocytes,
eosinophils and basophils

• First described by Alder in 1939 and


Reilly in 1941.
Pelger-Huet Anomaly

• Common inherited disorder that


shows hyposegmentation of
neucleus of segmented neutrophils.

• Nucleus maybe seen as peanut
shape or dumbbell shape.

• First described by Pelger in 1928


and Huet few years later.
Chediak-Higashi Syndrome
• Rare autosomal disorder showing giant
purple gray cytoplasmic granules of
neutrophils
• May also seen in lymphocytes.
• White blood cells are not fully functioning
and show reduced chemotaxis and
bactericidal killing function.
• Associated with albinism, organomegaly
and repeated infections
• Named after Dr. Moises Chediak (French
physician) and Ototaka Higashi
(Japanese physician) although this was
first described by Antonio Beguez-Cesar
(Cuban physician)
Job Syndrome
• Directional motility of phagocytes is
impaired but the random movement is
normal.

• Also calledHyperimmunoglobulin E
Syndrome.

• Name after biblical Job, whose


faithfulness was tested by an affliction
with draining skin sores and pustules.

• Patients with this condition have long-


term, severe skin infections.
Lazy Leukocyte Syndrome

• Both the random and directed motility of


the cells are defective.

• The condition is characterized by


recurrent stomatitis, otitis, gingivitis, and
low grade fever, with normal and cellular
immunity, however with severe
peripheral neutropenia.
Congenital C3 Deficiency

• rare inherited autosomal


recessive trait;

• failure of opsonization with


C3 are poorly recognized
and inefficiently
phagocytosed.
Chronic Granulomatous Disease

• a condition which
phagocytes ingest but
cannot kill catalase-positive
organisms because of the
lack of an appropriate
respiratory burst.
G6PD Deficiency

• absence of this enzyme


impairs the HMS and
phagocytes are unable to
produce respiratory burst
resulting in defective
bactericidal activity.
Myeloperoxidase Deficiency

• a relatively common
disorder inherited in an
autosomal recessive
fashion;

• a benign condition in
which patients are rarely
troubled by infections;
• without MPO bacterial
killing is slowed
incomplete
End of Lesson

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