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HEMATOLOGY 2 > PRELIM LECTURE L3: MYELOPROLIFERATIVE NEOPLASMS > Flashcards

PRELIM LECTURE L3: MYELOPROLIFERATIVE NEOPLASMS Flashcards

Ma'am Mitchao notes-based w/o table

1
Q

clonal hematopoietic disorder caused by genetic mutations in the HSC

A

myeloproliferative neoplasms

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2
Q

each myeloproliferative neoplasms is characterized by what cause

A

clonal expansion of one or more myeloid cell line

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3
Q

myeloproliferative neoplasms can progress into what condition

A

acute leukemia

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4
Q

4 predominant disorders of myeloproliferative neoplasms

A

chronic myeloid leukemia
polycythemia vera
essential thrombocytopenia
primary myelofibrosis

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5
Q

chronic myeloid leukemia is caused by what type of mutation

A

single genetic translocation in a pluripotent HSC

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6
Q

CML can progress to what disease if left untreated

A

acute leukemia (blast crisis phase)

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7
Q

age of CML

A

all, predominant in ages 46-53 years

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8
Q

CML represents how many percent of all cases of leukemia

A

20%

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9
Q

progression of CML can occur in what two types

A

myeloid type (AML)
lymphoid type (ALL)

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10
Q

T or F:
CML is more common in women than men

A

F
more common in men

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11
Q

symptoms of CML

A

fatigue
decrease tolerance of exertion
anorexia
abdominal discomfort
weight loss
splenic enlargement

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12
Q

present in proliferating HSCs and their progeny in CML; must be identified to confirm diagnosis

A

Philadelphia chromosome (Ph chromosome)

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13
Q

in 1960, Ph chromosome was determined as

A

short chromosome 22

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14
Q

who described Ph chromosome

A

Nowell and Hungerford

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15
Q

Ph is a reciprocal translocation between what chromosomes

A

long arm of chromosome 9 and 22

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16
Q

who discovered that Ph is a reciprocal translocation

A

Rowley, 1973

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17
Q

what gene mutated in CML

A

BCR-ABL1 gene

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18
Q

where does the translocation of BCR-ABL1 gene occurs

A

next to the SH3 domain of AB1 moiety

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19
Q

lab finding in CML caused by increased cell turnover

A

hyperuricemia and uricosuria

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20
Q

hyperuricemia and uricosuria may be associated with what conditions

A

secondary gout
uric acid stone
uric acid nephropathy

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21
Q

15% of px exhibit total WBC count of:

A

> 300 x 10^9/L

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22
Q

symptoms of CML are secondary to what causes

A

vascular stasis
intravascular consumption of oxygen by WBCs

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23
Q

useful for preliminary differentiation of CML from LR

A

Lap enzyme activity

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24
Q

increased cell lap enzyme activity indicates

A

Leukemoid reaction

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25
Q

decreased cell lap enzyme activity indicates

A

CML

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26
Q

first forms of therapy for CML

A

alkylating agents

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27
Q

Alkylating agents for CML

A

nitrogen mustard
busulfan in comibantion with 6-Thioguanine

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28
Q

other drugs for CML treatment

A

Hydroxyurea
6-mercaptopurine

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29
Q

treatment that dramatically improves outcomes of px with CML

A

interferon alpha

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30
Q

combined with interferon alpha that increases the frequency of px long term survival

A

Cytarabrine

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31
Q

polycythemia vera is aka

A

polycythemia rubra vera

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32
Q

a neoplastic clonal MPN

A

polycythemia vera

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33
Q

manifestations of polycythemia vera

A

panmyelosis in BM
increased RBC, granulocytes, platelets in PB
splenomegaly

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34
Q

polycythemia vera arises from what cell

A

HSC

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35
Q

incidence of polycythemia vera in Japan

A

2 cases/million

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36
Q

incidence of polycythemia vera in AUS and EU

A

13 cases/million

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37
Q

T or F:
PV is more common in men than women

A

T

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38
Q

T or F:
PV is more common in Jews

A

T

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39
Q

PV occurs often in what age

A

40-60 years

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40
Q

gene mutated in PV

A

JAK2 gene

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41
Q

what enzyme is JAK2 protein

A

tyrosine kinase

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42
Q

clinical presentation of PV

A

increase RBC mass
high hct (>60%)
hyperviscosity of blood produces
hypertension in 50% of px
headache
weakness
pruritis
weight loss
fatigue
thrombocytosis (half of px)
thrombotic or hemorrhagic episodes (1/3 of px)

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43
Q

thrombosis related events in PV

A

myocardial infarction
retinal vein thrombosis
thrombophlebitis
cerebral ischemia

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44
Q

stable phase of PCV can progress to what phase within 10 years of diagnosis

A

spent phase

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45
Q

clinical manifestations in spent phase

A

splenomegaly/hypersplenism
BM hyperplasia
pancytopenia

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46
Q

triad of PV

A

BM fibrosis
splenomegaly
anemia w/ teardrop shape poikilocyte

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47
Q

early stage PV treatment of choice

A

therapeutic phlebotomy

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48
Q

other treatments for PV

A

low dose of aspirin
alkylating agents

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49
Q

alkylating agent for high-risk px

A

hydroxyurea

50
Q

substitute alkylating agent for younger px with PV

A

interferon gamma

51
Q

alkylating agent for elderly with PV who develop intolerance or resistance to hydroxyurea

52
Q

a clonal MPN with increased megakaryopoiesis and thrombocytosis

A

essential thrombocythemia

53
Q

essential thrombocythemia is aka

A

primary thrombocytosis
idiopathic thrombocytosis
hemorrhagic thrombocythemia

54
Q

essential thrombocythemia has usually a count of

A

> 600 x 10^9/L, sometimes >1000 x 10^9/L

55
Q

count of sustained thrombocytosis required by WHO

A

> /= 400 x 10^9/L

56
Q

incidence rate of essential thrombocythemia

A

0.6-2.5 cases per 100,000 people per year

57
Q

prevalence rate of essential thrombocythemia

A

38-57 out of 100,000 people

58
Q

T or F:
essential thrombocythemia is more common with women than men

59
Q

major cases of essential thrombocythemia occur in what age

A

50-60 years

60
Q

second peak of essential thrombocythemia occurs in what age of women

A

childbearing years (30 years old)

61
Q

three mutations that are considered driver mutation for ET

A

JAK2 (64.1%)
MPL (4.3%)
CALR (15.5%)

62
Q

px with ET that are negative for all three mutation are referred to as

A

triple negative

63
Q

clinical presentations of ET

A

elevated platelet count
vascular occlusion
splenic atrophy
neurologic complications
arterial thrombi
bleeding that often occurs from mucous membranes

64
Q

arterial thrombi in ET can cause

A

MI
transient ischemic attack
cerebral vascular accident

65
Q

mucous membranes where bleeding often occurs in ET

A

GI
skin
urinary
URT

66
Q

neurologic complications in ET

A

headache
paresthesis of the extremities
visual impairments
tinnitus

67
Q

essential thrombocythemia must be differentiated from what conditions

A

reactive thrombocytosis and other MPN

68
Q

identification of which genes exclude the cases of reactive thrombocytosis

A

JAK VC17F and MPL W515K/L

69
Q

WHO requires how many major and minor criteria for ET diagnosis

A

4 major, 1 minor

70
Q

criteria for ET diagnosis

A

megakaryocyte proliferation w/ large and mature morphology
little to no granulocytes or erythroid proliferation
grade 1 reticulin fibers
must not meet any critera for BCR-ABL1 positive MPN and MDS
must demonstrate JAK2 VC17F, CALR, or MPL mutations

71
Q

minor criterion for ET

A

presence of clonal markers or absence of reactive thrombocytosis evidence

72
Q

platelets in ET can appear normal but can be accompanied with

A

giant bizarre platelet
platelet aggregates
micro megakaryocytes
megakaryocyte fragments

73
Q

leukocyte count in ET

A

22-40 x 10^9/L (leukocytosis)

74
Q

T or F:
neutrophils are normal in ET

A

F
may be inc

75
Q

other clinical findings in ET

A

presence of metamyelocytes and myelocytes
mildly elevated basophils and eosinophils
BM hypercellularity

76
Q

treatment for ET

A

plateletpheresis
alkylating agents

77
Q

alkylating agents for ET

A

hydroxyurea
anagrelide

78
Q

what can be done for px with intolerance or resistance to hydroxyurea

A

cytoreduction:
interferon gamma for younger px
busulfan for older px

79
Q

clonal HSC MPN where there is splenomegaly and ineffective hematopoiesis

A

primary myelofibrosis

80
Q

primary myelofibrosis is previously known as

A

chronic idiopathic myelofibrosis
agnogenic myelofibrosis
myelofibrosis with myeloid metaplasia

81
Q

areas of marrow hypercellularity (leukoerythroblastosis)

A

extramedullary hematopoiesis
fibrosis
increased megakaryocytes

82
Q

megakaryocytes are enlarged with

A

pleomorphic nuclei
coarse segmentation
areas of hypochromia

83
Q

disruption of normal BM architecture in PMF is caused by

A

over production of collagen

84
Q

myelofibrosis in PMF consists of how many types of collagens

85
Q

types of collagens in PMF

A

type I, III, IV

86
Q

percentage of JAK2 V617F in PMF

87
Q

percentage of MPL in PMF

88
Q

percentage of CLR in PMF

89
Q

percentage of TET2 in PMF

A

7.7-17% of px

90
Q

percentage of ASXL1 in PMF

A

13-23% of px

91
Q

percentage of EZH2 in PMF

92
Q

percentage of CBL in PMF

93
Q

percentage of LNK in PMF

A

3-6% of px

94
Q

percentage of IDH1/2 in PMF

A

4.2% of px

95
Q

genes in PMF

A

JAK2 V617F
MPL
CALR
TET2
ASXL1
DNMT3A
EXH2
CBL
LNK
IDH1/2

96
Q

PMF occurs in what age

97
Q

T or F:
PMF occurs more often in women than men

A

F
equally often

98
Q

T or F:
PMF manifestation can be asymptomatic or rapid

99
Q

symptoms of PMF resulted from anemia, meyloproliferation and splenomegaly

A

fatigue
weakness
shortness of breath
palpitation
loss of appetite
weight loss
night sweats
pruritis
pain in extremities and bones
bleeding
splenomegaly

100
Q

peripheral blood and bone marrow findings in PMF

A

quantitative and qualitative abnormalities
leukocytosis with left shift
thrombocytosis
fibrosis
pancytopenia
leukoerythroblastosis
anisocytosis
poikilocytosis

101
Q

treatment for severe anemia in PMF

A

androgen therapy
prenidisone
danazol
thalidomide
lenalidomide

102
Q

treatment for hemolytic anemia in PMF

A

glucocorticosteroids

103
Q

MPN that is a clonal disorder with neutrophil hyperproliferation

A

chronic neutrophilic leukemia

104
Q

chronic neutrophilic leukemia must be differentiated from

A

CML
myelodysplasia
reactive neutrophilic process

105
Q

incidence of chronic neutrophilic leukemia

A

rare, only 150 cases reported

106
Q

median age of diagnosis in chronic neutrophilic leukemia

107
Q

T or F:
male and female are equally affected in chronic neutrophilic leukemia

108
Q

most common clinical presentation in chronic neutrophilic leukemia

A

hepatosplenomegaly

109
Q

clinical presentations of chronic neutrophilic leukemia

A

fatigue
weight loss
easy bruising
bone pain
night sweats
bleeding from mucocutaneous sites e.g. GI tract (25-30%)
gout
pruritus

110
Q

wbc count in chronic neutrophilic leukemia

A

25 x 10^9/L

111
Q

neutrophil relative count in chronic neutrophilic leukemia

112
Q

findings in peripheral blood and bone marrow in chronic neutrophilic leukemia

A

increase band, metamyelocyte, myelocyte, and promyelocyte
hypercellular BM with predominantly proliferating neutrophils

113
Q

myeloid to erythroid ratio in chronic neutrophilic leukemia

114
Q

chromosomal abnormalities in chronic neutrophilic leukemia

A

+8, +9, +21
del (20q)
del (11q)
del (12p)

115
Q

first line of therapy in CNL

A

hydroxyurea, followed by interferon alpha

116
Q

therapeutic response in CNL lasts for how many months

117
Q

only curative treatment for CNL

A

allogenic stem cell transplant

118
Q

prognosis of CNL

A

slow, smoldering condition

119
Q

px survival with CNL

A

6 mos to more than 20 years

120
Q

median survival of px with CNL

121
Q

when CNL progresses to an accelerated phase and unresponsive to treatment, it exhibits

A

progressive neutrophilia
anima
thrombocytopenia
splenomegaly

HEMATOLOGY 2 (7 decks)

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