Disorders of Granulocytes and Monocytes

Question 1

It refers to the increase in
the concentration or
percentage of any of the
leukocytes in the peripheral blood

Answer 1

Leukocytosis

Question 2

It refers to the decrease in
the concentration or
percentage of any of the
leukocytes in the peripheral blood

Answer 2

Leukopenia

Question 3

is done to measure the total number
of white blood cells (granulocytes and
agranulocytes) in your blood

Answer 3

Total wbc count

Question 4

total wbc count diluent

Answer 4

3% acetic acid solution

Question 5

Normal WBC count

Answer 5

3.6 to 10.6 x 10⁹/L

Question 6

Adult Reference Value*
Relative count:

neutrphil -
lymphocyte -
monocyte -
eosinophil -
basophil -

Answer 6

neutrphil - 55% to 70%
lymphocyte -20% to 35%
monocyte - 3% to 8%
eosinophil - 1% to 3%
basophil - 0.5% to 1%

Question 7

Adult Reference Value*
Absolute count:

neutrphil -
lymphocyte -
monocyte -
eosinophil -
basophil -

Answer 7

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

Question 8

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

Answer 8

WBC differential count

Question 8

Normal WBC count

Answer 8

3.6 to 10.6 x 10⁹/L

Question 9

is done to correct the increased
WBC count due to the presence of nucleated RBCs

Answer 9

Corrected WBC count

Question 10

When is WBC count correction done?

Answer 10

If more than five (5) nucleated RBCs are seen per 100 WBCs classified

Question 11

Leukocytosis, depending on
the cause, may be classified as ____ or ____ leukocytosis

Answer 11

• physiologic
• non-malignant

Question 12

____ and ____ are present at birth and the few
days after birth, during labor and delivery

Answer 12

Physiologic leukocytosis
and neutrophilia

Question 13

This is neutrophilia in response to
physiologic or pathologic processes
(infection, inflammation, or malignancy)

Answer 13

Reactive Neutrophilia (Leukemoid Reaction)

Question 14

Increased demand for neutrophils will result
in the appearance of more immature
neutrophils in the peripheral or circulating
blood; this is called

Answer 14

“left shift” or “shift to the left”

Question 15

The LAP (leukocyte alkaline phosphatase)
score is high in ____ but is low in ____

Answer 15

• reactive state
• CML

Question 16

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

Answer 16

Leukemoid reaction

Question 17

Quality control: the acceptable LAP score of the known positive sample is

Answer 17

≥140

Question 18

The normal LAP score is established by the laboratory:

Answer 18

15 to 130 (Wintrobe’s Clinical Hematology, )

Question 19

Normal LAP score

Answer 19

~18 to 185 (may vary)

Question 20

(DISORDERS)

DECREASED LAP SCORES:

Answer 20

✓ Chronic myeloid leukemia
✓ Paroxysmal nocturnal
hemoglobinuria
✓ Sickle cell anemia
✓ Hereditary hypophosphatasia

Question 21

It is considered to exist if the number of
basophils exceeds 0.075 x 109/L

Answer 21

Basophilia

Question 22

It is a significant increase in circulating monocytes

Answer 22

Monocytosis

Question 23

The most common cause of leukopenia (granulocytopenia) is the decrease
in _____ , the major leukocyte type

Answer 23

segmented neutrophil

Question 24

Morphological Abnormalities of Mature Granulocytes

Answer 24

Nuclear Abnormalities Cytoplasmic Abnormalities

Question 25

are reported in increased numbers in inflammatory and neoplastic disorders in humans

Answer 25

Pyknotic neutrophils

Question 26

are purple or dark-blue staining azurophilic granules in the cytoplasm of band (or stab) and segmented neutrophils

Answer 26

Toxic granulation

Question 27

are light blue-gray, oval, basophilic, leukocyte inclusions (see arrows) located in the peripheral cytoplasm of neutrophils

Answer 27

Dohle bodies

Question 28

is the formation of vacuoles in the cytoplasm of neutrophils in response to severe infections or inflammatory conditions

Answer 28

Toxic vacuolization

Question 29

is a benign, dominantly inherited defect of terminal neutrophil differentiation secondary to mutations in the lamin B receptor (laminopathy)

Answer 29

PHA

Question 30

May be associated with a variety of conditions such as viral infections, burns, or certain drugs

Answer 30

Dohle bodies

Question 31

A genetic condition characterized by the presence of 2-5 μm basophilic, elongated Döhle body-like inclusions in granulocytes and monocytes

Answer 31

MAY-HEGGLIN ANOMALY

Question 32

▪ A rare autosomal recessive genetic disorder of granulocytes ▪ Primarily seen in children and young adults

Answer 32

CHÉDIAK-HIGASHI SYNDROME

Question 33

▪ 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

Answer 33

ALDER-REILLY ANOMALY

Question 34

Quantitative Leukocyte Disorders

Answer 34

- Defective Locomotion and Chemotaxis - Defective Microbicidal Activity

Question 35

It is a genetic mutation characterized by recurrent infections, neutropenia, abnormal neutrophil random (chemokinesis) and directed (chemotaxis) migration with defective neutrophil mobilization

Answer 35

LAZY LEUKOCYTE SYNDROME

Question 36

are characterized by the inability of leukocytes bind to vascular wall and to emigrate from the circulation to the sites of injury/inflammation

Answer 36

LEUKOCYTE ADHESION DEFICIENCY

Question 37

Defect: cytokine activation

Answer 37

LAD III

Question 38

(defective fucosylation); ▪ Defect: rolling

Answer 38

LAD II

Question 39

Defect: tight adhesion and emigration

Answer 39

LAD I

Question 40

acts by causing endothelial cells to release cyclic adenosine monophosphate (cAMP), which in turn interrupts adherence

Answer 40

Epinephrine

Question 41

In addition to leukocyte adhesion deficiency (LAD), adhesion defects can also be caused by two common drugs:

Answer 41

, epinephrine and corticosteroids

Question 42

▪ 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.

Answer 42

CGD

Question 43

▪ 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)

Answer 43

Nitroblue Tetrazolium (NBT) Test

Question 44

▪ 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

Answer 44

MYELOPEROXIDASE DEFICIENCY

Question 45

Defective Locomotion and Chemotaxis (disorders)

Answer 45

- Lazy Leukocyte Syndrome - Leukocyte Adhesion Deficiency - Drug-Induced Adhesion Defects

Question 46

Defective Microbicidal Activity (disorders)

Answer 46

CML - Chronic Granulomatous Disease - Myeloperoxidase Deficiency - Lactoferrin Deficiency

Question 47

Monocyte–Macrophage Disorders (QUALITATIVE)

Answer 47

- Lipid Storage Diseases - Mucopolysaccharide Storage Diseases

Question 48

Mucopolysaccharide Storage Diseases

Answer 48

- Hunter syndrome - Hurler Syndrome - Sea Blue Histiocytosis

Question 49

Lipid Storage Diseases

Answer 49

GNT - Gaucher Disease - Niemann-Pick Disease - Tay-Sachs Disease

Question 50

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

Answer 50

NIEMANN-PICK DISEASE

Question 51

▪ 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

Answer 51

TAY-SACHS DISEASE

Question 52

▪ Deficiency of the enzyme -glucocerebrosidase ▪ As a result of enzyme deficiency, glucocerebroside accumulates within the lysosomes of macrophages

Answer 52

GAUCHER DISEASE

Question 53

also known as inherited lipemic splenomegaly, is an extremely rare condition characterized by elevated triglyceride levels (hypertriglyceridemia) and an enlarged spleen (splenomegaly)

Answer 53

SEA BLUE HISTIOCYTOSIS

Question 54

▪ 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

Answer 54

HUNTER SYNDROME

Question 55

▪ 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

Answer 55

HURLER SYNDROME

Question 56

Hunter syndrome is caused by a deficiency of the lysosomal enzyme ____

Answer 56

iduronate-2-sulfatase enzyme