Leukopoiesis, WBC Differentiation, and Lymphocyte Function Flashcards

1
Q

How many WBCs are produced daily?

A

1.5 Billion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What stem cells give rise to WBCs?

A

Myeloid stem cells and lymphoid stem cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Myeloid stem cells and lymphoid stem cells originate from?

A

Pluripotent stem cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

The WBC maturation cycle is more complex than the RBC maturation cycle? T/F

A

True

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

How many WBCs are produced for 1 erythrocyte?

A

5 (4)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Neutrophils, Basophils, Eosinophils, and Monocytes are produced by what type of stem cell?

A

Myeliod stem cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Lymphoid stem cells give rise to?

A

B cells and T cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Where are B and T cells produced?

A

Bone marrow and thymus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

The word granulocytic applied to?

A

Only granulated WBCs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Which of the following applies to WBCs of all stages? a. Myelocytic b. Leukocytic c. Granulocytic d. All of the above

A

b. Leukocytic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Which of the following is synonymous with granulocytic? a. Myelocytic b. Leukocytic c. Granulocytic d. All of the above

A

b. Myelocytic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What do the majority of leukocytes contain?

A

Granules with enzymes used for digestion and destruction.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Where are Leukocytes located?

A

Bone Marrow, Circulation, Tissues, Some storage in the spleen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

These defend against bacteria, viruses, fungi, and foreign substances.

A

Leukocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Hematopoeisis is?

A

Process of cell production that maintains leukocytes, erythrocytes and platelets (3 diff cell lines)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Describe Leukopoiesis?

A

Process of generating WBCs (leukocytes)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Distinguish between marginating and circulating pools of leukocytes?

A

Circulating Pool - in the bloodstream Marginating Pool - in the vessel endothelium (inner layer of blood vessels)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

The morphology of what features are used to differentiate WBCs?

A
  1. Cell Size
  2. N:C ratio

3 . Chromatin pattern (presence/absence of nucleoli)

  1. Cytoplasmic quality (presence/absence of granules AND types of granules present).
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What are the granulocytes?

A

Eosinophils Basophils Neutrophils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What are the agranulocytes?

A

Monocytes

Lymphocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What are the 5 stages of (precursor) maturation from immature to most mature for neutrophils, basophils and eosinophils?

A
  1. Myeloblast N:C 6:1
  2. Promyelocyte N:C 3:1
  3. Myelocyte N:C 2:1
  4. Metamyelocyte N:C 1:1
  5. band / eosinophilic band / basophilic band

Final Form: Segmented neutrophil / basophil / eosinophil

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What are the stages of Monocytic Maturation?

A
  1. Monoblast N:C 6:1
  2. Promonocyte N:C 3:1
  3. Monocyte N:C 1:1
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What are the 3 stages of Lyphocytic Maturation?

A
  1. Lymphoblast N:C 4:1
  2. Prolymphocyte N:C 3:1
  3. Small Lymphocyte N:C 4:1 / Large Lymphocyte N:C 3:1
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Describe eosinophilic granules?

A

Round, large, dirty orange

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Describe basophilic granules?

A

large, dark blue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Describe Neutrophilic granules?

A

Very fine, pinkish color and begin near nucleus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

At what stage can granulocytes be distinuished?

A

Myelocyte stage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What is the function of neutrophils?

A

Seek, ingest, kill bacteria phagocytocis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What is the most numerous WBC?

A

Neutrophils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What is the function of eosinophils?

A

Respond to allergic and parasitic reactions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

What is the function of basophils? What do they contain?

A

Respond to hypersensitivity and reactions and inflammation

Contain: Herparin and hystamine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

What are the primary Lymphoid organs?

A

Bone Marrow and thymus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

What are the secondary organs of the lymphatic system? (4)

A

Spleen, Peyer’s patches, lymph nodes, tonsils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

What are the responsibilities of the lymphatic system?

A

Fluid balance

Lymphopoiesis

Antibody generation

Blood filtration

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Describe lymph fluid?

A

Thin, clear, derived from plasma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

How is lymp circulated?

A

Circulates from respiration

muscle movement

nearby vessel pressure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

What are the primary locations of lymphopoiesis?

A

Bone marrow: B cells

Thymus: T cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

What lymphoid stem cells migrate to the thymus? (2)

A

T helper cells

T supressor cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

What lymphiod cells stay in bone marrow?

A

B cells

NK cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

60 - 85% of lymphocytes

Cell mediated immunity

mature in the thymus

A

T Cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Once stimulated by antigen contact T cells?

A

CD4 (T helper cells) help promote antibody production and assist with immune intracellular pathogens

CD8 (T killer cells) cytotoxic elimination of non-self by promoting enzyme activity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

10 - 20 % of lymphocytes

Mature into plasma cells that produce anibodies

Humoral immunity

may be stimulated toward a particular immune response

A

B Cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

<20% of lymphocytes

role in resisting bacteria, viruses and fungi

do not require stimulation

A

Natural Killer (NK) cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

Foreign bodies emit signals sensed by neutrophils

These signals are used to move towards the site of invasion

A

Chemotaxis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Neutrophil receptors attach to foreign body. this attachedment is enhanced after body has been opsonized with complement or Igs

A

Opsonization

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Opsonized foreign body is _____ by nuetrophil and ______

Neutrophilic granules ______ _______ which contain elements that destroy foreign body and the neutrophil too.

A

ingested, killed

release contents

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

Leukocytosis

A

Absolute increase in total WBC count

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

What is a Left Shift?

A

Exaggerated response to infection and inflammation, which may include:

  • toxic vaculozation
  • Dohle bodies
  • toxic granulation
  • immature cells younger than metamyelocyte but rarely blasts
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

What is Leukoerythroblastic picture?

A

Peripheral smear contains immature WBCs, nRBCs, and platelet abnormalities.

Significant feature of myeloproliferative disorders

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

In which stage of neutrophilic maturation are specific secondary granules first seen?

A

Myelocyte

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

These following condistions cause what quantitative change in which WBC line?

Infections (bacterial)

Inflammatory response

Stress response

Malignancies

chemical assault

A

Neutrophilia (increase)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

These following condistions cause what quantitative change in which WBC line?

Drugs

chemotherapy

autoimmune disease

A

Neutropenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

These following condistions cause what quantitative change in which WBC line?

allergies

skin disease

parasitic disease

transplant rejection

myeloproliferative disorders

A

Eosinophila (increase)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

These following condistions cause what quantitative change in which WBC line?

Acute infections

ACTH

Bone Marrow Aplasia

A

Eosinopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

These following condistions cause what quantitative change in which WBC line?

Myelo proliferative disorders

hypersensitivity reactions

Ulcerative colitis

Chronic inflammatory conditions

A

Basophila (increase)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

These following condistions cause what quantitative change in which WBC line?

Steriod treatment

inflammation

A

Basopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

These following condistions cause what quantitative change in which WBC line?

Chronic infections (TB)

Malignancies

Leukemias w/ stong monocytic component

bone marrow failure

A

Monocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

These following condistions cause what quantitative change in which WBC line?

Autoimmune processes

Hairy cell leukemia

A

Monocytopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

These following condistions cause what quantitative change in which WBC line?

Normal in children 4 - 4 months old

Viral (CMV, EBV, HIV)

Leukemias

A

Lymphocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

These following condistions cause what quantitative change in which WBC line?

HIV

malnutrition

chemotherapy

radiation

renal failure

A

Lymphocytopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

Infection, Human Erlichiosis, and Megaloblastic processes are _______ conditions causing qualitative WBC changes.

A

Acquired

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q
  • May-Hegglin Anomaly
  • Alder-Reily Anomaly
  • Palger-Huet Anomaly
  • Chediak-Higashi Syndrome
  • Lipid Storage Diseases

The above are all __________ conditions causing qualitative WBC changes.

A

Hereditary

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

Left shift:

  • increase in bands and metamyelocytes in the peripheral smear.
  • larger than normal basophilic granules within the cytoplasm of neutrophils, bands, and metamyelocytes. Resemble primary granules or promyelocytes

Toxic vacuolization:

  • Round, clear spaces within the granulite cytoplasm
  • Dohle bodies
  • round, oval light blue staining found in the cytoplasm
  • Remnants of RNA
A

Infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q
  • Notable white cell inclusion cause by two varieties of Rickettsia-like bacteria:
    • Erlichia chafeenis
    • Anaplasma phagocytophilum
  • Common to both illnesses is low WBC count, elevated liver enzymes, and thrombocytopenia. Inclusions may be seen in the granulocyte or monoytes from the bone marrow.
A

Human Erlichiosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q
  • Hypersegmentation
  • Folic acid deficiency
  • Pernicious anemia
  • B12 deficiency
A

Megaloblastic Processes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
66
Q
  • Autosomal Dominant
  • Thrombocytopenia and giant platelets (poorly granualated)
  • Larger Dohle bodies found in cytoplasm of neutrophils
A

May-Hegglin Anomaly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
67
Q
  • Rare genetic disorder
  • Prominent depostion of granules in every cell line
  • Lipid deposition in the cytoplasm
A

Alder-Reilly Anomaly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
68
Q
  • Most common (dominant trait)
  • 70 - 95% hyposegementation
  • Heterozygotes: dumbell shaped nuccleus
  • Homozygotes: nucleus is spherical
  • Pseudo - _____ _____ - myeloproliferative disorders, severe infections and leukemias.
A

Pelger-Huet Anomaly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
69
Q
  • Rare autosomal recessive disorder of neutrophilic granuels
  • giant purple grey cytoplasmic granuels
  • lymphocytes and monocytes nay show a signle red granule in cytoplasm
  • WBCs show reduced chemotaxis and bactericidal killing function
  • hepatospenomegaly and liver failure may develop
  • abnormal bleeding time
  • Affected children may show albinism and photophobia
A

Chediak-Higashi Syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
70
Q

Lipid storage diseases are the result of?

A

a missing or inactive strategic enzyme

caused by a singele gene deletion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
71
Q
  • deficiency of the enzyme beta-glucocerebrosidase
  • leads to accumulation of glucocerebrosidase mostly in monocytes /macrophages
  • characterized by severe bone pain
A

Gaucher’s disease

(accumution causes Gacher cells)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
72
Q
  • Deficiency of the enzyme sphingomyelinase
  • enlarged liver and spleen
  • thrombocytopenia
  • sea-blue histocytes in bone marrow
  • macrophages have globular cytoplasm
A

Neimann-Pick Disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
73
Q
  • deficiency of the enzyme hexaminosidase A
  • No large identifiable bone marrow cells
  • can be tested prenatally
  • deafness and blindness
  • seizure
  • death in a few years
A

Tay-Sachs Disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
74
Q

Normal for young children 1 - 4 years

Differential will show reversal of lymphocytes to segmented neutrophils

normal morphology

A

Relative lymphocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
75
Q

Reactive Lymphocytosis represents?

A

a response to viral infection such as EBV and CMV

76
Q

What is the most common disease showing variation in lymphocytes?

A

Mononucleosis

77
Q

Infectious mononucleosis is caused by _______ _______, which infects __ __________.

A

Epstein-Barr Virus

B Lymphocytes

78
Q

What are some symptoms of mononucleosis?

A

Sore throat

fatigue

anorexia

fever

enlarged lymph nodes

79
Q

What 2 ways is mononucleosis diagnosed?

A

rapid agglutination tests or careful examination of smear

80
Q

Reactive lymphocytes (ATL) charateristics?

A

Scallop red cells and abundant royal blue cytoplasm

81
Q

What virus is endmic worldwide?

A

CMV

82
Q

What is CMV isolated from?

A

respiratory secretions

urine

semen

cervical secretions

83
Q

_______ of blood donors show anti-CMV titres?

A

40 - 90%

84
Q

Donor blood for infants must be ?

A

CMV Negative

85
Q

Seen in lipid storage disease, macrophage with globular cytoplasm

A

Nieman Pick Cell

86
Q

change can be seen during serious infections (2)

A

Toxic granulation

Toxix vacuolization

87
Q

seen in lipid storage disease, crinkled tissue paper appearance of cytoplasm

A

Gaucher cell

88
Q

Seen in megaloblastic anemias

A

Hypersegmented neutrophils

89
Q

WBC reaction can be seen in mononucleosis?

A

Reactive Lympocytosis

90
Q

giant-grey green cytoplasmic granules

A

Chediak-Higashi

91
Q

Giant platelets and Dohle bodies in cytoplasm

A

May-Hegglin Anomaly

92
Q

Hyposegmentation seen in the majority of neutrophils

A

Pelger-Huet Anomaly

93
Q

What is the most common leukemia affecting adults?

A

AML

94
Q

In an AML smear what would be seen?

Blasts

Auer rods

Pseudo Pelger-Huet cells

possible NRBCs*

All of the above

A

All of the above

95
Q

What is the prognosis for AML?

A

fatal in 2-3 months w/o treatment

96
Q

What acute leukemia affects children <10 and peaks between 2- 6 years old?

A

ALL

97
Q

T/F the peripheral smear in ALL is:

Lab: elevated WBC count

close to 100% lymphoblasts and lymphocytes

A

True

98
Q
A

ALL Prognostic factors

99
Q

What are the 4 main WHO groups of AMLs?

A
  • AML w/ recurrent cytogenetic abnormalities
  • AML w/ myelodsplasia
  • Therapy related AMLs and MDS
  • AML not otherwise specificied
100
Q

What is the WHO requires what blast percentage?

A

>20% in blood or bone marrow

101
Q

What is Dysplasia?

A

the abnormal maturation of cells in bone marrow

102
Q

What dysplastic features are seen in AML w/ Myelodysplasia?

  • Platelets
A

May exhibit micromegakaryocyte with one lobe instead of multiple lobes

103
Q

What dysplastic features are seen in AML w/ Myelodysplasia?

  • Erythroid
A

Nucleated red cells

megaloblastic features

cytoplasmic vacuoles

ringed sideroblast

104
Q

What dysplastic features are seen in AML w/ Myelodysplasia?

  • Neutrophils
A

Hypogranulation

Hyposegmentation or Pseudo-pelgeriod

Bizzarre segmented neclei

105
Q

What are the 4 WHO classfications on AML that do not fit into other catogories and is primarity based on morphology and cytochemistry?

A

AML, w/ Maturation

Acute Monoblastic and Monocytic Leukemia

Acute Erythroid Leukemia

Acute Lymphoblastic Leukemia

106
Q

A clinical feature of acute leukemia is leukemic cells?

A

Ovewhelm bone marrow

  • Anemia (normocytic-normochromic)
  • Thrombocytopenia
107
Q

AML Not otherwise specified:

  • >80% monocytic origin
  • ________ predominance of blasts
  • ________ predominance of promonocytes
A

AML, w/ maturation

Acute monoblastic leukemia

Acute monocytic leukemia

108
Q

AML Not otherwise specified

  • The most common leukemia: 30-45% of AML
  • >10% maturation beyond promyelocyte
  • monocytic compenent <20% noneythroid cells
A

AML, w/ maturation

109
Q

A hallmark clinical feature is extramedullary disease and ginigival hypertrophy

A

Acute monoblastic and acute monocytic leukemia

110
Q

AML Not otherwise specified:

  • Abnormal proliferation of erthroid precursors
  • >50% or BM cells are erthroid and at least 30% are myeloblast
  • Pure _____ leukemia is >80% precursors
A

Acute Erthroid leukemia

Erythroid

111
Q

AML Not otherwise specified:

  • most rare form of the AMLs
  • at least 20% of blasts in BM are megakaryoblasts
A

Acute Megakaryoblastic Leukemia

112
Q

What are the two WHO groups of Lymphoblastic Leukemias?

A
  • Precusor B cell
  • Precursor T cell
113
Q

Lymphoblastic Leukemia occurs in _____ ____ and _____

  • _____ of BM cells are lymphoblast

Lymphoblastic Lymphoma occurs in _____ _____

  • ______ _____ and _____ of BM are lymphoblast
A

BM and blood, > 25%

Lymph tissue, mass lesion and <25%

114
Q

AML with recurrent Genetic abnormailities:

  • Monoblasts and promonocytes in peripheral blood and BM
  • often show pseudopodia
  • fine nuclear chromatin with 1 or more nuclei
  • azurophilic granules
  • cytoplasmic vacuoles
A

AML w/ 11q23

115
Q

AML with recurrent Genetic abnormailities:

  • hypergranualar promyelocytes
  • multiple auer rods
  • some mocrogranular promyleocytes
  • assocuated w/ DIC
A

Acute promyelocytic leukemia t(15:17)

116
Q

AML with recurrent Genetic abnormailities:

  • hypergranualar promyelocytes
  • multiple auer rods
  • some mocrogranular promyleocytes
  • assocuated w/ DIC
A

Acute promyelocytic leukemia t(15:17)

117
Q

AML with recurrent Genetic abnormailities:

  • increased eosinophils
  • varios stages of myelocytic, monocytic, and eosinophilic maturation
  • high remission rates
A

AML inv (16)

118
Q

AML with recurrent Genetic abnormailities:

  • Large myeloblast
  • auer rods common
  • young WBC seen in smear
  • eosinophils increased
  • good long-term survival
A

AML t(8:21)

119
Q

What are Chronic Myeloprolferative DIsorders (CMPDs)?

A

The are a group disorders that are clonal malignancies (disease arising from single cell line).

120
Q

What are the cell lines affected in these CMPDs?

  • CML
  • PV
  • IMF
  • ET
A
  • Granulocytic
  • Erythrocytic
  • Fibroblast/All
  • Megakaryocytic
121
Q

What is the age of onset for CML?

A

20 - 50 years

122
Q

CML shows marked neutrophil leukocytocis, with a WBC amouting?

A

WBC >50,000

123
Q

90 - 95% of CML patients have what chromosome?

Explain how it is created and what gene results from this mutation, and the effect it has.

A

Philedelphia (Ph) Chromosome

Results from translocation from chromosome 22 to chromosome 9 forming BRA-ABL fusion gene. This gene results in increased tyrosine kinase activity, which prevents apoptosis, creating an immortal cell line. (excess cell prolifereation and production)

124
Q

Describe the clinical features of CML? (3)

A

Can be asymptomatic

Normocytic/normochromic anemia

3 Phases: chronic, accelerated, and blast.

125
Q

The M:E ratio in CML can be as high as?

A

25:1

(10:1 in BM)

126
Q

Describe the Peripheral Blood and Bone Marrow for the Chronic phase of CML?

A

PB

  • blasts >2%
  • throbocytosis

BM

  • blast <5%
  • M:E ratio 10:1
127
Q

Describe the Peripheral Blood and Bone Marrow for the Blast phase of CML?

A

PB

  • blasts >20%
  • throbocytopenia

BM

  • blast>20%
  • Large clusters of blasts
  • Marked dysplasia on all 3 cell lines
128
Q

Describe the Peripheral Blood and Bone Marrow for the Accelerated phase of CML?

A

PB

  • basophils >20%
  • throbocytopenia

BM

  • Dysplasia
  • Fibrosis
129
Q

Leukocyte Alkaline Phosphatase (LAP) differentiates CML from a leukemoid reaction, what is the LAP reference range and score in CML?

A

Ref 20 - 100

CML <13

130
Q

3 Treatments for CML?

A
  • Myelosupressive surgery to inhibit BCR-ABL tyrosine kinaase
  • Leukophoesis - cell seperator to decrease WBC count
  • Allogenic BM transplant
131
Q

CML has a poor prognosis based on what factors? (6)

A
  • age
  • phase of CML
  • amount of blasts in peripheral blood
  • size of spleen
  • marrow fibrosis
  • general health
132
Q
A
133
Q

T/F

There is no treatement for acute CML?

A

True

134
Q

Which phase of CML is reponsive to treatment?

A

Chronic phase

135
Q

What is the age of onset for Polycythemia Vera?

A

60 - 70 years old

136
Q

Describe the BM in PV?

A

Hypercellular with Hyperplasia

137
Q

What is PV? and what does it lead to?

A

Clonal disorder: overproductions of mature RBCs, WBCs, and PLTs

increaed Hgb, HCT, and RBC mass which increases blood viscosity and predisposition to arterial and venous thrombosis

138
Q

What mutation is present in PV and what percentage of patients have it?

A

90% JAK2 mutation

139
Q

What does the JAK2 mutation do?

A

activates a kinase that sends a signal to cell to proliferate leading to increased RBCs.

140
Q

Clinical features of PV are

  • Major symptoms related to and increase in red cell mass, which are? (3)
  • Increased blood cell turnover (1)
  • Abdominal pain (1)
A
  • Hypertension, hyperviscosity, vascular abnormalities
  • headache, light headedness, blurred-vision
  • thrombosis
  • Hyperuricemia, gout, stomach ulcers
  • hepatomegaly / splenomegaly
141
Q
A
142
Q

What are 2 diagnostic characteristics of PV?

A
  • Increase in all 3 cell lines
  • Neutrophilia with shift to the left and basophili
  • LAP score elevated
  • Serum erythropoetin low compared to secondary erthrythocytosis
143
Q

What is the most significant diagnostic finding in PV that distinguishes it from secondary and relative erythropoiesis (3)?

A
  • Increase in Red Call Mass
  • Splenomegaly w/ increase in leukocytes and platelets
  • JAK2 mutation
144
Q

What is the goal of treatment in Polycythemia Vera?

A

decrease HCT

145
Q

What are treatments for Polycythemia Vera? (2)

A
  • Therapeutic Phlebotomy
  • Low dose apsrin to manage thrombosis risk
146
Q

Which chronic myeloproliferative disorder has a median survival time of 10 years?

A

Polycythemia Vera

147
Q

What is Idiopathic myelofobrosis (IMF) is charaterized by?

A

Bone marrow fibrosis, proliferation of megakaryocytic, granulocytic and extramedullary disease.

148
Q

Which CMPD is characterized by extramedullary disease?

A

Idiopathic Myelofibrosis (IMF)

149
Q

What are 3 clinical features of Idiopathic Myelofibrosis (IMF)?

A
  • Early stages asymptomatic
  • Pancytopenia
  • Osteosclerosis
150
Q

A typical peripheral smear in Idiopathic Myelofibrosis (IMF) will have what RBC?

A

Tear drop

151
Q

How is Idiopathic Myelofibrosis (IMF) diagnosed? (6)

A
  • CBC
  • Spleomegaly
  • BM fibrosis
  • Leukoeythroblastic picture
  • No increase in red cell mass
  • no philedelphia chromosome
152
Q

T/F

There are no treatments to reverse the process of myelofibrosis?

A

True

153
Q

Hydroxyurea is used in Idiopathic Myelofibrosis (IMF) as a cytoreductive therapy to control? (3)

A
  • Leukocytosis
  • Thrombocytosis
  • Organomegaly
154
Q

Which CMPD has the worst prognosis? Survival?

A

Idiopathic Myelofibrosis (IMF)

3-5 years

155
Q

What is Essential Thrombocythemia?

A

CMPD charaterized by disorder of multipotenial stem cell

156
Q

Essential Thrombocythemia can affect ___ ____ cell lines.

A

All 3 cell lines

157
Q

In what cell line is the main increase in Essential Thrombocythemia?

A

Megakaryocytes

158
Q

How does Essential Thrombocythemia affect PLTs?

A

Increase in PLTs due to hypersensitivity of megakaryocytes to cytokines and thrombin

159
Q

What is a hallmark diagnostic criteria of Essential Thrombocythemia?

A

Unexplained elevated PLT count

160
Q

Hydroxyurea, anagrelide, or alfa-interferon are used in treament of Essential Thrombocythemia to ? (2)

A
  • reduce PLT count
  • decrease risk of vaso-occlusion or hemmorhage
161
Q

What treament is used in severe cases of Essential Thrombocythemia?

A

plateletpheresis

162
Q

What is the survival rate for Essential Thrombocythemia?

A

10 years for 64 - 80%

163
Q

Most patients with Essential Thrombocythemia die from?

A

thrombotic complications

164
Q

Clonal Lyphoproliferative disorders primarily affect what age group?

A

Elderly

165
Q

Clonal Lymphoproliferative Disorders are ?

A

Clonal malignant proliferation of B and T lymphocytes

166
Q

Clonal Lymphoproliferative Disorders are diagnosed by? (2)

A
  • Flow cytometry
  • chromosoml analysis / molecular
167
Q

Chronic Lymphocytic Leukemia (CLL) is clonal proliferation of?

A

B lymphocytes

168
Q

Caused by chromosomal abnormalities

Small lymhocytes accumulate in the spleen, lymph nodes, bone marrrow, and can spill out into peripheral curculation.

A

Clonal Lymphocytic Leukemia (CLL)

169
Q

Clonal Lymphocytic Leukemia (CLL) Peripheral smear is? (2)

A
  • nearly exclusively small lymphocytes with few lymphoblasts
  • smudge cells present
170
Q

Clonal Lymphocytic Leukemia (CLL) Treatment?

A
  • Irradiation or enlarged spleen and lymph nodes
  • Drugs to reduce lymphocyte burden
171
Q

Clonal Lymphocytic Leukemia (CLL) mature lymphs are?

A

non-fucntional

172
Q

In 80% of Clonal Lymphocytic Leukemia (CLL), what gene is present?

A

anti-apoptosis gene BCL 2

173
Q

hypogammaglobinemia is commin in?

A

Clonal Lymphocytic Leukemia (CLL)

174
Q

What is a rare B cell malignancy?

A

Hairy Cell Leukemia

175
Q

Hairy Cell Leukemia - what causes a dry tap?

A

BM becomes filled with fibrotic material and BM cannot be aspirated.

176
Q

What stain is used in diagnosis of Hairy Cell Leukemia?

What happens when stained?

A

Tartrate-resistant acid phosphatase (TRAP)

Hairy cells are strongly posisitive and stay positive tartrate acid is added, while other clls become negative.

177
Q

What is Sezary Syndrome?

A

T cell lymphoma w/ mycosis fungoides

178
Q

Sezary Syndrome manifests?

A

Cutaneous manifestation

179
Q

What cell is being described?

  • Large cells
  • Oviod, clefted or folded nucleus
  • Very smooth homogenous chromatin pattern
  • May be mistaken for monocytes
A

Sezary Cells

180
Q

Describe cause of Hodgkin’s Lymphoma? how is it diagnosed?

A

Singel cervical lymph node becomes firm to touch and does not dissapear,

Lymph node biopsy

181
Q

What cell is being described, where is it located, and which cancer is it unique to?

  • Large
  • multinucleated
  • resembles “owls eye”
A

Reed-Sternberg cell in bone marrow

Hodgkin’s lymphoma

182
Q

Non-hodgkins lymphoma is is 3X more common than hodgkin’s and does not have Reed-Sternberg Cells?

T/F

A

True

183
Q

Plasma cell evolves from?

A

B lymphocyte

184
Q

What are the 3 pathways of Multiple Myeloma?

A
  1. Acceleration of plasma cells in BM
  2. Activation of bone reabsorbtion factors or Osteoclasts
  3. Production of an abnormal monoclonal protein
185
Q

In what stage of Multiple Myeloma will the following present:

  • May develop colorless inclusions called Russell bodies or other crystalline inclusions
  • Flame cells may be seen in IgA myeloma
A

Acceleration on plasma cells in BM

186
Q

In what stage of Multiple Myeloma will the following present:

  • increase in osteoclast activity
    • bone loss
      *
A