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Hematology > Disorders of Granulocytes and Monocytes > Flashcards

Disorders of Granulocytes and Monocytes Flashcards

1
Q

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

A

Leukocytosis

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2
Q

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

A

Leukopenia

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3
Q

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

A

Total wbc count

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4
Q

total wbc count diluent

A

3% acetic acid solution

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5
Q

Normal WBC count

A

3.6 to 10.6 x 10⁹/L

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6
Q

Adult Reference Value*
Relative count:

neutrphil -
lymphocyte -
monocyte -
eosinophil -
basophil -

A

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

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7
Q

Adult Reference Value*
Absolute count:

neutrphil -
lymphocyte -
monocyte -
eosinophil -
basophil -

A

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

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8
Q

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

A

WBC differential count

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8
Q

Normal WBC count

A

3.6 to 10.6 x 10⁹/L

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9
Q

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

A

Corrected WBC count

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10
Q

When is WBC count correction done?

A

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

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11
Q

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

A
  • physiologic
  • non-malignant
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12
Q

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

A

Physiologic leukocytosis
and neutrophilia

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13
Q

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

A

Reactive Neutrophilia (Leukemoid Reaction)

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14
Q

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

A

“left shift” or “shift to the left”

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15
Q

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

A
  • reactive state
  • CML
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16
Q

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

A

Leukemoid reaction

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17
Q

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

A

≥140

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18
Q

The normal LAP score is established by the laboratory:

A

15 to 130 (Wintrobe’s Clinical Hematology, )

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19
Q

Normal LAP score

A

~18 to 185 (may vary)

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20
Q

(DISORDERS)

DECREASED LAP SCORES:

A

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

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21
Q

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

A

Basophilia

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22
Q

It is a significant increase in circulating monocytes

A

Monocytosis

23
Q

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

A

segmented neutrophil

24
Q

Morphological Abnormalities of Mature Granulocytes

A

Nuclear Abnormalities
Cytoplasmic Abnormalities

25
Q

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

A

Pyknotic neutrophils

26
Q

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

A

Toxic granulation

27
Q

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

A

Dohle bodies

28
Q

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

A

Toxic vacuolization

29
Q

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

A

PHA

30
Q

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

A

Dohle bodies

31
Q

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

A

MAY-HEGGLIN ANOMALY

32
Q

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

A

CHÉDIAK-HIGASHI SYNDROME

33
Q

▪ 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

A

ALDER-REILLY ANOMALY

34
Q

Quantitative Leukocyte Disorders

A
  • Defective Locomotion and Chemotaxis
  • Defective Microbicidal Activity
35
Q

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

A

LAZY LEUKOCYTE SYNDROME

36
Q

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

A

LEUKOCYTE ADHESION DEFICIENCY

37
Q

Defect: cytokine activation

A

LAD III

38
Q

(defective fucosylation);
▪ Defect: rolling

A

LAD II

39
Q

Defect: tight adhesion and emigration

A

LAD I

40
Q

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

A

Epinephrine

41
Q

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

A

, epinephrine and corticosteroids

42
Q

▪ 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.

A

CGD

43
Q

▪ 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)

A

Nitroblue Tetrazolium (NBT) Test

44
Q

▪ 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

A

MYELOPEROXIDASE DEFICIENCY

45
Q

Defective Locomotion and Chemotaxis (disorders)

A
  • Lazy Leukocyte Syndrome
  • Leukocyte Adhesion Deficiency
  • Drug-Induced Adhesion Defects
46
Q

Defective Microbicidal Activity (disorders)

A

CML

  • Chronic Granulomatous Disease
  • Myeloperoxidase Deficiency
  • Lactoferrin Deficiency
47
Q

Monocyte–Macrophage Disorders (QUALITATIVE)

A
  • Lipid Storage Diseases
  • Mucopolysaccharide Storage Diseases
48
Q

Mucopolysaccharide Storage Diseases

A
  • Hunter syndrome
  • Hurler Syndrome
  • Sea Blue Histiocytosis
49
Q

Lipid Storage Diseases

A

GNT

  • Gaucher Disease
  • Niemann-Pick Disease
  • Tay-Sachs Disease
50
Q

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

A

NIEMANN-PICK DISEASE

51
Q

▪ 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

A

TAY-SACHS DISEASE

52
Q

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

A

GAUCHER DISEASE

53
Q

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

A

SEA BLUE HISTIOCYTOSIS

54
Q

▪ 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

A

HUNTER SYNDROME

55
Q

▪ 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

A

HURLER SYNDROME

56
Q

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

A

iduronate-2-sulfatase enzyme

Hematology (4 decks)

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