Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Lindsey's Blood and Lymph Unit III > Myelodysplastic Syndrome & Myeloproliferative Neoplasms > Flashcards

Myelodysplastic Syndrome & Myeloproliferative Neoplasms Flashcards

1
Q

What does the BCR-ABL1 fusion protein do?

A

constitutive activation of several growth pathways

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

Who gets secondary erythrocytosis?

A

smokers; people with chronic hypoxia, certain hemoglobin disorders

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

What is plethora?

A

dusky, reddish skin

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

How is MDS diagnosed?

A

at least 1 persistent cytopenia; evaluate bone marrow and chromosomes

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

What is CML?

A

a hematopoietic stem cell disorder assoc. with BCR-ABL1 gene fusion; usually neutrophilic leukocytosis

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

How is CML diagnosed?

A

presence of BCR-ABL1 via cytogenetics, FISH, or RT-PCR of mRNA

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

Which disease is indolent?

A

ET

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

Why does hepatomegaly occur in MPN?

A

extramedullary hematopoeisis

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

Most cases of PV contain the ____ mutation.

A

V617F point

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

PMF eventually progresses to the ____ stage, in which the bone marrow has reticulin fibrosis, the peripheral blood has leukoerythroblastosis, and other organs enlarge because of extramedullary hematopoiesis.

A

fibrotic

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

How is ET diagnosed?

A

incidentally bc of increased platelet counts

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

What is the incidence rate of CML? Avg. age at diagnosis?

A

1-2/100,000; 40-50yo

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

What is the outcome of CML?

A

80-85% survival

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

What is ET?

A

an MPN characterized by sustained thrombocytosis and no granulocytic hyperplasia in the marrow

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

What does PMF stand for?

A

primary myelofibrosis

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

What are paresthesias?

A

pins-and-needles sensation

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

In the prefibrotic stage of PMF, the marrow is hypercellular, showing a _____ and _____ proliferation.

A

granulocytic; megakaryocytic

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

What is the prognosis of PMF? Survival?

A

poor if diagnosed in fibrotic stage- median survival 5 years

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

What is the prognosis of ET?

A

good; survival 10-15 years or more; an indolent disease

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

What is RC-UD?

A

cytopenia of the one of the various lineages (ie anemia, neutropenia)

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

In the ____ stage of PV, peripheral blood cell counts are increased.

A

polycythemic

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

What does MDS stand for?

A

myelodysplastic syndrome

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

What are the 2 broad clinical scenarios of MDS?

A
  1. 1a/idiopathic 2. 2a/therapy related (t-MDS)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What does PV stand for?

A

polycythemia vera

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

What karyotypes can be seen in MDS?

A

monosomy or deletion of 5 or 7; trisomy 8

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

What are MPNs?

A

proliferations of 1+ myeloid lineages

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

The JAK2 mutation can be found in ____ of cases of PMF.

A

50%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is the treatment for CML?

A

PTKIs: imatinib (Gleevac) and dasatinib

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

What would be found in a marrow biopsy of CML in the chronic phase?

A

hypercellularity; granulocytic hyperplasia; small megakaryocytes with round, non-lobated nuclei; no dysplasia

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

Once MDS has been diagnosed, it is classified as either ____ or ____.

A

low grade; high grade

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

PMF eventually progresses to the fibrotic stage, in which the bone marrow has ____, the peripheral blood has _____, and other organs enlarge because of ____.

A

reticulin fibrosis; leukoerythroblastosis; extramedullary hematopoiesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

PV should always be considered in a pt with a thrombosis of the ____, ____, or _____ vein.

A

mesenteric, portal, splenic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What are the s/s of PV?

A

thrombosis of the mesenteric, portal, or splenic vein; headaches; dizziness; visual probs; paresthesias; plethora; itching; splenomegaly; hepatomegaly

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

Before diagnosing MDS, you should rule out non-neoplastic causes of 2a myelodysplasia: ____, ______, _____, and _____.

A

certain drugs (chemo); B12, folic acid, and other deficiencies; viral infection; heavy metal (arsenic) and other toxin exposures

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

What is PV?

A

an MPN characterized by increased RBC mass (erythrocytosis) accompanied by increased neutrophils and platelets (trilineage hyperplasia)

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

What are ring sideroblasts?

A

RBCs that have iron accumulated in the mitochondria that encircle the nucleus- stains blue

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

What does dysgranulopoiesis look like?

A

nuclear hypolobation; neutrophils with bilobed nuclei called pseudo-Pelger-Huet cells; hypogranularity

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

What are the 2 types of high grade MDS?

A
  1. refractory anemia with excess blasts-1 (RAEB-1) 2. refractory anemia with excess blasts-2 (RAEB-2)
27
Q

What is the initial phase of CML, and what are its characteristics?

A

the chronic phase; normal blasts, increased neutrophils, basophils, and (usu) platelets

27
Q

What is PMF?

A

proliferation of the granulocytic and megakaryocytic lineages with eventual progression to myelofibrosis

28
Q

MPNs are usually associated with abnormalities of genes (translocations, point mutations) that encode _____.

A

cytoplasmic or receptor protein tyrosine kinase (PTKs)

30
Q

Untreated MPNs lead to _____, _____, and _____.

A
  1. AML or ALL 2. myelodysplasia with ineffective hematopoiesis (MDS) 3. marrow fibrosis and failure
31
Q

What is common to all MPNs?

A

increase in 1+ cell types; hypercellular marrow; spleno- or hepatomegaly; insidious onset usually detected by CBC

32
Q

What is the prognosis for refractory anemia with excess blasts-1 (RAEB-1)? Median survival? Transformation?

A

poor; median survival 16 mos; 25% of patients transform to AML

34
Q

Dysplastic changes in at least ____ % of cells in one or more lineages is diagnostic for MDS.

A

10

35
Q

What is the bone marrow like in ET?

A

atypical megakaryocytes are even larger and more bizarre than PMF

36
Q

____ mutations can be found in 50% of pts with ET.

A

JAK2

38
Q

What is the incidence rate of MPNs?

A

6-10/100,000

39
Q

What is seen on the PV bone marrow biopsy?

A

trilineage hyperplasia; clusters of large, bizarre megakaryocytes

40
Q

What constitutes the BCR-ABL1 gene fusion?

A

translocation (9;22)(q34;q11.2) on the derivative chromosome 22 (Philadelphia chromosome)

41
Q

What is the most serious complication of PV?

A

venous or arterial thrombi

42
Q

What does dysmegakaryopoiesis look like?

A

megakaryocytes have hypolobated or non-lobated nuclei; hyperchromatic nuclei; small size

42
Q

How are PV and PMF different?

A

no erythrocytosis in PMF

42
Q

Where does extramedullary hematopoiesis occur?

A

spleen, liver, lymph nodes

43
Q

____ eventually progresses to the fibrotic stage, in which the bone marrow has reticulin fibrosis, the peripheral blood has leukoerythroblastosis, and other organs enlarge because of extramedullary hematopoiesis.

A

PMF

44
Q

Who gets 1a/idiopathic MDS, and what is the incidence rate?

A

people over 50; avg age at diagnosis is 70; 3-5/100,000

45
Q

In the prefibrotic stage of PMF, the marrow is _____, showing a granulocytic and megakaryocytic proliferation.

A

hypercellular

47
Q

What is the 2nd phase of CML? What are its characteristics?

A

the blast phase; essentially acute leukemia (>20% blasts in the blood)

48
Q

Who gets 2a/t-MDS?

A

persons exposed to alkylating agents or IR 2-8 years later

49
Q

Which cells make up the blast phase in CML?

A

myeloblasts (70%) and lymphoblasts (30%)

51
Q

What does RC-UD stand for?

A

refractory cytopenia with unilineage dysplasia

53
Q

Who gets MPNs?

A

adults 50-70yo

54
Q

What is the later stage of PV called, and what characterizes it?

A

spent phase; prominent fibrosis of the marrow and falling peripheral blood cell counts

56
Q

What does high grade MDS mean?

A

myeloblasts account for >5% of marrow cells and/or more than 2% of peripheral blood cells

56
Q

What is the prognosis of RC-MD? Survival rate? Transformation?

A

poor- median survival 2.5 years- 10% transformation to AML

58
Q

What is RC-MD?

A

low grade MDS with dysplasia in 2 or more lineages

59
Q

In the ____ stage of PMF, the marrow is hypercellular, showing a granulocytic and megakaryocytic proliferation.

A

prefibrotic

61
Q

What is the prognosis for refractory anemia with excess blasts-2 (RAEB-2)? Median survival? Transformation?

A

poor; 9 mos; 33% of patients transform to AML

62
Q

What are the treatments of PV?

A

serial phlebotomy; aspirin; mild chemo

64
Q

What is MDS with isolated deletion 5q?

A

low grade MDS assoc. with anemia, increased platelets, and megakaryocytes with small, round, non-lobated nuclei

65
Q

What is refractory anemia with excess blasts-1 (RAEB-1)?

A

5-9% blasts in the marrow or 2-4% blasts in the peripheral blood

67
Q

Nearly all cases of PV have a mutation of the _____ gene that encodes a _____.

A

JAK2; JAK2 cell signaling protein

69
Q

What is the intermediate phase of CML?

A

the accelerated phase

70
Q

What are the 4 main types of MPNs?

A
  1. chronic myelogenous leukemia (CML) 2. polycythemia vera (PV) 3. primary myelofibrois (PMF) 4. essential thrombocytopenia (ET)
71
Q

What are the 3 types of low grade MDS?

A
  1. refractory cytopenia with unilineage dysplasia (RC-UD) 2. refactory cytopenia with multilineage dysplasia (RC-MD) 3. MDS with isolated deletion 5q
73
Q

What does ET stand for?

A

essential thrombocytopenia

74
Q

What does RC-MD stand for?

A

refactory cytopenia with multilineage dysplasia

75
Q

What does MPN stand for?

A

myeloproliferative neoplasms

76
Q

What does dyserythropoeisis look like?

A

RBC precursors with nuclear budding, irregular shaped nuclei, lack of coordinated nuclear/cytoplasmic maturity, increased ring sideroblasts

77
Q

What is refractory anemia with excess blasts-2 (RAEB-2)?

A

10-19% blasts in the marrow or 5-19% blasts in the peripheral blood

78
Q

What are dacrocytes?

A

tear drop RBCs

80
Q

What are the s/s of CML?

A

fatigue, weight loss, night sweats, splenomegaly, anemia

81
Q

What is the prognosis of RC-UD?

A

relatively good

82
Q

What are the s/s of ET?

A

TIAs; digital ischemia with parasthesias; thrombosis of major BVs

84
Q

What does low grade MDS mean?

A

myeloblasts account for

86
Q

What is the treatment for MDS?

A

stem cell transplant, supportive care, transfusions

87
Q

What is the prognosis of PV? Survival?

A

very good- 10-20 years

88
Q

What is MDS?

A

hematopoietic stem cell disorder characterized by ineffective hematopoiesis and increased risk of transformation to acute myeloid leukemia (AML)

89
Q

____ should always be considered in a pt with a thrombosis of the mesenteric, portal, or splenic vein.

A

PV

90
Q

MPNs are usually associated with _____ that encode cytoplasmic or receptor protein tyrosine kinase (PTKs).

A

abnormalities of genes (translocations, point mutations)

91
Q

What is the prognosis of CML?

A

poor- median survival 2-3 years

92
Q

The ____ mutation can be found in 50% of cases of PMF.

A

JAK2

93
Q

What does CML stand for?

A

chronic myelogenous leukemia

Lindsey's Blood and Lymph Unit III (10 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions