Disorders of Granulocytes and Monocytes Flashcards
It refers to the increase in
the concentration or
percentage of any of the
leukocytes in the peripheral blood
Leukocytosis
It refers to the decrease in
the concentration or
percentage of any of the
leukocytes in the peripheral blood
Leukopenia
is done to measure the total number
of white blood cells (granulocytes and
agranulocytes) in your blood
Total wbc count
total wbc count diluent
3% acetic acid solution
Normal WBC count
3.6 to 10.6 x 10⁹/L
Adult Reference Value*
Relative count:
neutrphil -
lymphocyte -
monocyte -
eosinophil -
basophil -
neutrphil - 55% to 70%
lymphocyte -20% to 35%
monocyte - 3% to 8%
eosinophil - 1% to 3%
basophil - 0.5% to 1%
Adult Reference Value*
Absolute count:
neutrphil -
lymphocyte -
monocyte -
eosinophil -
basophil -
neutrphil - 1.7 to 7.5
lymphocyte - 1.0 to 3.2
monocyte - 0.1 to 1.3
eosinophil - 0.0 to 0.3
basophil - 0.0 to 0.2
is done to measure the number
(concentration and percentage) of each white blood cell type in your blood. Immature WBCs and nucleated RBCs may be seen during the counting process; their presence must be noted in the worksheet
WBC differential count
Normal WBC count
3.6 to 10.6 x 10⁹/L
is done to correct the increased
WBC count due to the presence of nucleated RBCs
Corrected WBC count
When is WBC count correction done?
If more than five (5) nucleated RBCs are seen per 100 WBCs classified
Leukocytosis, depending on
the cause, may be classified as ____ or ____ leukocytosis
- physiologic
- non-malignant
____ and ____ are present at birth and the few
days after birth, during labor and delivery
Physiologic leukocytosis
and neutrophilia
This is neutrophilia in response to
physiologic or pathologic processes
(infection, inflammation, or malignancy)
Reactive Neutrophilia (Leukemoid Reaction)
Increased demand for neutrophils will result
in the appearance of more immature
neutrophils in the peripheral or circulating
blood; this is called
“left shift” or “shift to the left”
The LAP (leukocyte alkaline phosphatase)
score is high in ____ but is low in ____
- reactive state
- CML
an increase in the white blood cell count (leukocytosis), which can mimic leukemia.
The reaction is actually due to an infection (“left-shift”) or another disease and is not a sign of cancer
Leukemoid reaction
Quality control: the acceptable LAP score of the known positive sample is
≥140
The normal LAP score is established by the laboratory:
15 to 130 (Wintrobe’s Clinical Hematology, )
Normal LAP score
~18 to 185 (may vary)
(DISORDERS)
DECREASED LAP SCORES:
✓ Chronic myeloid leukemia
✓ Paroxysmal nocturnal
hemoglobinuria
✓ Sickle cell anemia
✓ Hereditary hypophosphatasia
It is considered to exist if the number of
basophils exceeds 0.075 x 109/L
Basophilia
It is a significant increase in circulating monocytes
Monocytosis
The most common cause of leukopenia (granulocytopenia) is the decrease
in _____ , the major leukocyte type
segmented neutrophil
Morphological Abnormalities of Mature Granulocytes
Nuclear Abnormalities
Cytoplasmic Abnormalities
are reported in increased numbers in inflammatory and neoplastic disorders in humans
Pyknotic neutrophils
are purple or dark-blue staining azurophilic granules in the cytoplasm of band (or stab) and
segmented neutrophils
Toxic granulation
are light blue-gray, oval, basophilic, leukocyte inclusions (see arrows) located in the peripheral
cytoplasm of neutrophils
Dohle bodies
is the formation of vacuoles in the cytoplasm of neutrophils in response to severe infections or
inflammatory conditions
Toxic vacuolization
is a benign, dominantly inherited defect of terminal neutrophil differentiation secondary to mutations in the lamin B receptor (laminopathy)
PHA
May be associated with a variety of conditions such as viral infections, burns, or certain drugs
Dohle bodies
A genetic condition characterized by the presence of 2-5 μm basophilic, elongated Döhle body-like inclusions in granulocytes and monocytes
MAY-HEGGLIN ANOMALY
▪ A rare autosomal recessive genetic disorder of
granulocytes
▪ Primarily seen in children and young adults
CHÉDIAK-HIGASHI SYNDROME
▪ It is commonly seen in the mucopolysaccharidoses such as Hurler’s, Hunter’s, and Maroteaux-Lamy syndrome
▪ It is an autosomal recessive inherited disorder
characterized by the presence of large purple-red
granules (surrounded by a clear halo) in cells of the myeloid and lymphocytic series
ALDER-REILLY ANOMALY
Quantitative Leukocyte Disorders
- Defective Locomotion and Chemotaxis
- Defective Microbicidal Activity
It is a genetic mutation characterized by recurrent infections, neutropenia, abnormal
neutrophil random (chemokinesis) and directed (chemotaxis) migration with defective
neutrophil mobilization
LAZY LEUKOCYTE SYNDROME
are characterized by the inability of leukocytes
bind to vascular wall and to emigrate from the
circulation to the sites of injury/inflammation
LEUKOCYTE ADHESION DEFICIENCY
Defect: cytokine activation
LAD III
(defective fucosylation);
▪ Defect: rolling
LAD II
Defect: tight adhesion and emigration
LAD I
acts by causing endothelial cells to release cyclic adenosine monophosphate (cAMP), which in turn interrupts adherence
Epinephrine
In addition to leukocyte adhesion deficiency (LAD), adhesion defects can also be caused
by two common drugs:
, epinephrine and corticosteroids
▪ It is the most serious disorder related to a defect in microbicidal activity
▪ It consists of a group of disorders in which neutrophils and monocytes ingest, but cannot
kill, catalase-positive microorganisms such as Staphylococcus aureus, Gram-negative
enteric bacteria, and various fungi, especially Aspergillus spp.
CGD
▪ Semi-quantitative assay
▪ is a blood test that measures the ability of the immune system to convert the colorless nitroblue tetrazolium (NBT) to a deep blue
▪ This test is performed as a screen for chronic
granulomatous disease (CGD)
Nitroblue Tetrazolium (NBT) Test
▪ Alius-Grignaschi anomaly
▪ A benign inherited disorder that is transmitted by autosomal recessive genes
▪ The disorder is manifested by the deficiency of the ______ enzyme from neutrophils and monocytes, but not eosinophils
MYELOPEROXIDASE DEFICIENCY
Defective Locomotion and Chemotaxis (disorders)
- Lazy Leukocyte Syndrome
- Leukocyte Adhesion Deficiency
- Drug-Induced Adhesion Defects
Defective Microbicidal Activity (disorders)
CML
- Chronic Granulomatous Disease
- Myeloperoxidase Deficiency
- Lactoferrin Deficiency
Monocyte–Macrophage Disorders (QUALITATIVE)
- Lipid Storage Diseases
- Mucopolysaccharide Storage Diseases
Mucopolysaccharide Storage Diseases
- Hunter syndrome
- Hurler Syndrome
- Sea Blue Histiocytosis
Lipid Storage Diseases
GNT
- Gaucher Disease
- Niemann-Pick Disease
- Tay-Sachs Disease
is a rare, autosomal recessive lysosomal storage disorder characterized by a deficiency of acid sphingomyelinase, leading to the accumulation of sphingomyelin within cells and tissues
NIEMANN-PICK DISEASE
▪ GM2 Gangliosidosis, Type 1
▪ is a rare, neurodegenerative
disorder in which deficiency of an enzyme
(hexosaminidase A) results in excessive
accumulation of certain fats (lipids) known as GM2 gangliosides in the brain and nerve cells
TAY-SACHS DISEASE
▪ Deficiency of the enzyme -glucocerebrosidase
▪ As a result of enzyme deficiency, glucocerebroside accumulates within the lysosomes of macrophages
GAUCHER DISEASE
also known as inherited lipemic
splenomegaly, is an extremely rare condition
characterized by elevated triglyceride levels
(hypertriglyceridemia) and an enlarged spleen
(splenomegaly)
SEA BLUE HISTIOCYTOSIS
▪ Mucopolysaccharidosis type II (MPS II), also known as ____ , is a condition that affects many
different parts of the body and occurs almost
exclusively in males
▪ is the only MPS syndrome to exhibit X-linked recessive inheritance
HUNTER SYNDROME
▪ formerly gargoylism
▪ Most severe form of mucopolysaccharidosis
▪ It is characterized by a deficiency of the enzyme
alpha-L-iduronidase, which results in an
accumulation of dermatan and heparan sulfates
HURLER SYNDROME
Hunter syndrome is caused by a deficiency of the lysosomal enzyme ____
iduronate-2-sulfatase enzyme
