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[LECT] HEMA 2 - Finals > M3: Myeloproliferative Neoplasms > Flashcards

M3: Myeloproliferative Neoplasms Flashcards

1
Q

Clonal hematopoietic _____ disorders that result in (excessive/decreased) production and overaccumulation of ______, ______, and _______

A

MYELOPROLIFERATIVE NEOPLASMS (MPNs)

stem cell
excessive
erythrocytes
granulocytes
platelets

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2
Q

Clonal hematopoietic stem cell disorders that result in excessive production and overaccumulation of erythrocytes, granulocytes, and platelets in some combination in ____, _____, and _____

A

MYELOPROLIFERATIVE NEOPLASMS (MPNs)

bone marrow
peripheral blood
body tissues

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3
Q

has something to do with the bone marrow and hematopoietic cells

A

Myelo

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4
Q

Myelo - has something to do with the ______ and ______

A

bone marrow
hematopoietic cells

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5
Q

could also refer to myeloid cell series

A

Myelo

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6
Q

Myelo - could also refer to _________

A

myeloid cell series

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7
Q

proliferate or production

A

Proliferative

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8
Q

an abnormal mass of tissue that forms when cells grow and divide more than they should or do not die when they should

A

Neoplasm

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9
Q

Neoplasm - an abnormal _____ that forms when cells grow and divide more than they should or do not ___ when they should

A

mass of tissue
die

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10
Q

FOUR MAJOR CONDITIONS (WHO)

A

Chronic myeloid leukemia (CML)
Polycythemia vera (PV)
Essential thrombocytopenia (ET)
Primary myelofibrosis (PMF)

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11
Q

What is CML

A

Chronic myeloid leukemia

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12
Q

What is PV

A

Polycythemia vera

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13
Q

What is ET

A

Essential thrombocytopenia

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14
Q

What is PMF

A

Primary myelofibrosis

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15
Q

Consistently associated with the BCR-ABL fusion gene located in the Philadelphia chromosome

A

Chronic Myelogenous Leukemia (CML)

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16
Q

Chronic Myelogenous Leukemia (CML)

Consistently associated with the _____ fusion gene located in the ____ chromosome

A

BCR-ABL
Philadelphia

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17
Q

reciprocal translocation of DNA between chromosomes 9 and 22

A

Ph chromosome

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18
Q

Ph chromosome – reciprocal translocation of DNA between chromosomes ___ and __

A

9
22

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19
Q

Large numbers of myeloid precursors in the bone marrow, peripheral blood, and extramedullary tissues

A

Chronic Myelogenous Leukemia (CML)

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20
Q

CML

exhibits leukocytosis with increased myeloid series, particularly the later maturation stages, often with increases in eosinophils and basophils

A

Peripheral blood

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21
Q

CML

Peripheral blood - exhibits ____ with (inc/dec) myeloid series, particularly the later maturation stages, often with increases in _____ and _____

A

leukocytosis
Increased
eosinophils
basophils

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22
Q

CML

score is dramatically decreases

A

Leukocyte alkaline phosphatase (LAP)

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23
Q

What does LAP stand for

A

Leukocyte alkaline phosphatase

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24
Q

Leukocyte alkaline phosphatase (LAP) score is dramatically (inc/dec)

A

decreases

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25
Q

An enzyme found in the membranes of secondary granules of neutrophils

A

Leukocyte Alkaline Phosphatase Stain (LAP)

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26
Q

LAP

An enzyme found in the membranes of ______ granules of ____

A

secondary
neutrophils

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27
Q

LAP

Purpose: stains __present in most ___

A

ALP
neutrophil

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28
Q

To differentiate CML (↓) from leukemoid reaction or polycythemia vera

A

Leukocyte Alkaline Phosphatase Stain (LAP)

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29
Q

LAP

To differentiate CML (inc/dec) from leukemoid reaction or _____

A

↓
polycythemia vera

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30
Q

↑ leukemoid reaction, _____, ____

A

polycythemia vera
3rd trimester of pregnancy

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31
Q

The degree of reactivity is measured by scoring each of 100 neutrophils according to the amount of precipitated dye present

A

Leukocyte Alkaline Phosphatase Stain (LAP)

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32
Q

LAP

The degree of reactivity is measured by scoring each of _______ according to the amount of precipitated ___ present

A

100 neutrophils
dye

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33
Q

Scored from 0 to 4 (strong)

A

LAP

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34
Q

LAP

Scored from ____ (strong)

A

0 to 4

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35
Q

Heparin must be used

A

LAP

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36
Q

LAP

___ must be used

A

Heparin

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37
Q

LAP

_____ – falsely decreased

A

EDTA

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38
Q

_____ exhibits intense hypercellularity with a predominance of myeloid precursors

A

CML

Bone marrow (BM)

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39
Q

Bone marrow (BM) exhibits intense _____with a predominance of ____

A

hypercellularity
myeloid precursors

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40
Q

______ numbers are normal to increased

A

CML

Megakaryocyte

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41
Q

Patients with CML can progress from a (chronic/acute) stable phase (gradual) through an _____ into transformation to (chronic/acute) leukemia (more rapid)

A

Chronic
accelerated phase
Acute

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42
Q

____ is the preferred approach to treatment CML;

A

BM Transplantation

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43
Q

BM Transplantation

Patients can also be administered with _____.

A

tyrosine kinase inhibitors

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44
Q

the predominant cell type is mature;

A

Chronic

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45
Q

the onset is insidious or gradual but with harmful effects

A

Chronic

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46
Q

Increased WBC count

A

Chronic

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47
Q

Chronic

Increased ___

A

WBC count

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48
Q

Production ins lower from months to years

A

Chronic

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49
Q

Chronic

Production ins lower from _____ to ___

A

months to years

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50
Q

type of leukemia that arises from cells of the myeloid series

A

Myelogenous:

51
Q

Aside from the presence of _____ proteins, CML also contains the excess production of _____ and one of the targets of CML is the _____

A

BCR-ABL
Tyrosine kinase
tyrosine kinase inhibitor

52
Q

an enzyme that promotes survival and proliferation of AML cells

A

Tyrosine kinase

53
Q

Tyrosine kinase: an enzyme that promotes survival and proliferation of ____

A

AML cells

54
Q

What does AML stand for?

A

Adult acute myeloid leukemia

55
Q

____________: it targets abnormal BCR-ABL protein to block its function causing the CML cells to die

A

Tyrosine kinase inhibitor

56
Q

Who are the person important for the discovery of PV

A

William Osler
Dameshek

57
Q

William Osler what year?

A

1910

58
Q

Dameshek what year?

A

1951

59
Q

Polycythemia Rubra Vera

A

William Osler (1910)

60
Q

Clinical description was that of a patient with engorged veins, plethora (swelling in the face and palms), and an elevated red blood cell count

A

William Osler (1910)

61
Q

William Osler (1910)

Clinical description was that of a patient with ___, ____(swelling in the face and palms), and an elevated ______

A

engorged veins
plethora
red blood cell count

62
Q

Leukocytosis and thrombocytopenia were recognized as additional features

A

William Osler (1910)

63
Q

William Osler (1910)

____and _____ were recognized as additional features

A

Leukocytosis
thrombocytopenia

64
Q

Added PV to the classification of MPNs

A

Dameshek (1951)

65
Q

Dameshek (1951)

Added PV to the classification of ____

A

MPNs

66
Q

Main differential diagnosis is that of reactive erythrocytosis due to hypoxia

A

Dameshek (1951)

67
Q

Dameshek (1951)

Main differential diagnosis is that of ______ due to _______

A

reactive erythrocytosis
hypoxia

68
Q

Dameshek (1951)

Presence of ______ = erythropoiesis is stimulated

A

hypoxia

69
Q

Dameshek (1951)

Inc. RBC makes the blood _____ than normal and may lead to ____ and other complication

A

thicker
blood clots

70
Q

PV is characterized by

Clonal stem cell disorder characterized by hyperproliferation of the ___, ____, and ________

A

erythroid
myeloid
megakaryocytic lineage

71
Q

requires that three major criteria and one minor criterion be met

A

WHO diagnostic criteria

72
Q

WHO MAJOR CRITERIA

  1. Hemoglobin _____ in men and ____ in women OR Hematocrit ____ in men and _____ in women OR (inc/dec) red blood cell (RBC) mass mean normal
A

> 16.5 g/dL OR >49% - MEN
>16.0 g/dL OR >48% - WOMEN
inc

73
Q

WHO MAJOR CRITERIA

  1. Bone marrow showing hypercellularity for age with trilineage growth (___) including prominent ____, ____, and _____ proliferation with ____, mature megakaryocytes (difference is size)
A

panmyelosis
erythroid
granulocytic
megakaryocytic
pleomorphic

74
Q

WHO MAJOR CRITERIA

  1. Presence of JAK2V617F or ________
A

JAK2 exon 12 mutation

75
Q

WHO MAJOR CRITERIA

  1. Presence of ______ or JAK2 exon 12 mutation
A

JAK2V617F

76
Q

WHO MAJOR CRITERIA

  1. Presence of ___ or _____
A

JAK2V617F
JAK2 exon 12 mutation

77
Q

there is a change in the normal structure of protein;

A

JAK2V617F or JAK2 exon 12 mutation

78
Q

there is a switch of amino acid ____ to _____ at position ______ which changes the shape of the JAK2 protein

A

JAK2V617F or JAK2 exon 12 mutation

Valine (V)
Phenylalanine (F)
617

79
Q

a protein important for controlling the production of blood cells from the hematopoietic stem cells

A

JAK (Janus kinase)

80
Q

regulator protein for the hematopoietic stem cell production

A

JAK (Janus kinase)

81
Q

WHO MINOR CRITERION

Subnormal _____ level

A

serum erythropoietin

82
Q

Additional diagnostic features of PV include

  1. Arterial oxygen saturation of _____ (normal) or greater and ___
A

92%
splenomegaly

83
Q

Additional diagnostic features of PV include

  1. Thrombocytosis of greater than _____
A

400 x 109 platelets/L

84
Q

Additional diagnostic features of PV include

  1. Leukocytosis of greater than ______ without fever or infection
A

12 x 109 cells/L

85
Q

Additional diagnostic features of PV include

  1. Increases in ___, ____, or _______ binding capacity
A

LAP
serum vitamin B12
unbound vitamin B12

86
Q

PV - Clinical Signs and Symptoms

Associated with increased RBC mass – _____

A

hyperviscosity

87
Q

PV - Clinical Signs and Symptoms

HCT ____ resulting to ____ in about 50% of patients

A

> 60%
hypertension

88
Q

PV - Clinical Signs and Symptoms

Presenting symptoms are associated with _____ and _____ and include headache, weakness, pruritis, weight loss, and fatigue

A

hyperviscosity
hyperproliferation

89
Q

_____ is a hallmark of PV

A

Phletora

90
Q

PV - Clinical Signs and Symptoms

_____ has been associated with elevated blood ___ and is often not associated with a ____

A

Pruritis
histamine
visible rash

91
Q

PV - Clinical Signs and Symptoms

About ____ of PV patients have ____ and ____
experience ______ or ____ episodes

A

half
thrombocytosis
one third
thrombotic or hemorrhagic episodes

92
Q

Laboratory Data of PV

__ erythrocyte count, packed cell volume, hemoglobin

A

increase

93
Q

What values are increased in the lab data of PV

A

erythrocyte count, packed cell volume, hemoglobin

94
Q

Laboratory Data OF PV

Normal ___

A

erythrocytic indices

95
Q

Laboratory Data OF PV

↓ _____

A

serum erythropoietin

96
Q

Why is serum erythropoietin is decreased in the lab data of PV

A

red cell proliferation is thought to be independent of endogenous erythropoietin

97
Q

t/f: red cell proliferation is thought to be dependent of endogenous erythropoietin

A

false; INDEPENDENT

98
Q

LAB DATA OF PV:

Peak polycythemic values:
→ Hemoglobin of approximately ___
→ Microhematocrit of approximately ___
→ Total leukocyte (white blood cell [WBC]) count
of ___
→ Platelet count of ____

A

20.6 g/dL

80%

28,000 × 109/L

1,400 × 109/L

99
Q

TREATMENT FOR PV

A

Therapeutic phlebotomy, myelosuppressive therapy, and targeted molecular therapy

100
Q

a controlled
removal a large volume of blood to reduce volume, red cell mass and iron stores

A

Therapeutic phlebotomy

101
Q

Characterized by a significant increase in circulating platelets,

A

Essential Thrombocytosis or Essential
Thrombocythemia (ET)

102
Q

Essential Thrombocytosis or

A

Essential
Thrombocythemia (ET)

103
Q

Essential Thrombocytosis or Essential
Thrombocythemia (ET) Characterized by a significant increase in circulating ____, usually in ___ of ____

A

platelets
excess
1,000 × 109/L

104
Q

WHO criteria require a sustained thrombocytosis with a platelet count of _____

A

400 x 109/L or greater

105
Q

In ET, Elevated ____ may be encountered as a ___, secondary to a variety of ____, or they may represent essential ___,

A

platelet counts
reactive phenomenon
systemic conditions
thrombocythemia

106
Q

primary disorder of the bone marrow

A

essential thrombocythemia

107
Q

Essential Thrombocytosis or Essential
Thrombocythemia (ET) is AKA

A

→ primary thrombocytosis
→ idiopathic thrombocytosis
→ hemorrhagic thrombocythemia

108
Q

WHO diagnostic criteria include the following: (ET)
* MAJOR CRITERIA
1. ___ proliferation with ___ and ___ morphology, little to no _____ or ____ proliferation

  1. Rarely, minor (___) ___ in ____
  2. Must not meet any criteria for ____-positive ___, ___, ___, ____ or other ____
  3. Must demonstrate ____, ___, or ___ mutations
A
  1. Megakaryocyte; large; mature ; granulocyte ; erythroid
  2. grade 1; increase; reticulin fibers
  3. BCR-ABL1; CML; PV; PMF; MDS; myeloid neoplasms
  4. JAK2 V617F; CALR; MPL
109
Q

WHO diagnostic criteria include the following (for ET)

*MINOR CRITERIA
presence of a ___ or absence of evidence of ___

A

clonal marker

reactive thrombocytosis

110
Q

PB and BM in ET shows:

● Platelets often appear ___, but giant ___ platelets, platelet ___, ____, and ____ fragments can also be observed

A

normal;

bizarre

aggregates

micromegakaryocytes

megakaryocyte

111
Q

PB and BM in ET shows:

Erythrocytes are ____ and ___, unless ___ is present secondary to excessive ___

A

normocytic

normochromic

iron deficiency

bleeding

112
Q

PB and BM in ET shows:

Splenic infarction can result in ___, nucleated ____, and ___

A

Howell-Jolly bodies

erythrocytes

poikilocytosis

113
Q

Treatment for ET:

Treatment is initiated to ___ the ____ and control ___

A

reduce

platelet count

thrombosis

114
Q

can be used to quickly reduce the platelet count

A

Plateletpheresis

115
Q

Plateletpheresis uses an ____ machine that only removes ___ and then returns the other components of blood to the ___.

A

apheresis

platelets

body

116
Q

Results when primary myelofibrosis progeny cells stimulate bone marrow fibroblasts to secrete excessive collagen

A

Primary Myelofibrosis (PMF)

117
Q

Primary Myelofibrosis (PMF) Results when primary ___ progeny cells stimulate bone marrow ___ to secrete excessive ___

A

myelofibrosis

fibroblasts

collagen

118
Q

In PMF, Overproduction of collagen eventually
___ the ___ architecture of the ___ and replaces ___ tissue resulting in ___

A

disrupts;

normal

BM

hematopoietic

pancytopenia

119
Q

decreased production in all types of cells

A

pancytopenia

120
Q

In primary myelofibrosis, nucleated red blood cells (___) and ___ are released into the circulation (___) when there is ___ hematopoiesis (i.e., ___have taken over blood cell production because of the ___ marrow)

A

normoblasts

myelocytes

leukoerythroblastosis

extramedullary

non-marrow organs

fibrosed

121
Q

Diagnosis of Primary Myelofibrosis:
MAJOR CRITERIA

● ___ proliferation with ____ morphology, usually accompanied by ___ and/or ___
● Not meeting the criteria for other ___
● Evidence of ___ or other related ___

A

Megakaryocytic; abnormal; reticulin; collagen fibrosis

MPNs

JAK2V617F; mutations

122
Q

Diagnosis of Primary Myelofibrosis
MINOR CRITERIA

● ____ (presence of nucleated red cells and immature white cells)
● ___
● ____ (Increased/Decreased) ____ levels
●___

A

Leukoerythroblastosis

Anemia

increased; serum lactic dehydrogenase (LDH)

Splenomegaly

123
Q

presence of nucleated red cells and immature white cells

A

Leukoerythroblastosis

124
Q

nucleated red blood cells aka

A

normoblasts

[LECT] HEMA 2 - Finals (1 decks)

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