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WILLIAM'S HEMATOLOGY > 83 Polycythemia Vera > Flashcards

83 Polycythemia Vera Flashcards

1
Q

The term denoting an increased amount of blood cells that has traditionally been applied to conditions in which the mass of erythrocytes is increased

A

Polycythemia

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2
Q

TRUE OR FALSE

In polycythemia vera, there is more than one cell lineage increased at diagnosis and through the course of the untreated disease, from erythrocytosis, an invariably unilineage increase in red cells

A

TRUE

In polycythemia vera, there is more than one cell lineage increased at diagnosis and through the course of the untreated disease, from erythrocytosis, an invariably unilineage increase in red cells

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3
Q

A characteristic feature of PV on BMA

A

Endogenous erythroid colonies (EECs)

Erythropoietin-independent BFU-E precursors

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4
Q

A situation wherein a part of a chromosome comes in two copies from one parent and no copy from the other parent.

A

Uniparental disomy (UPD)

In PV, UPD is the acquired somatic event generated by mitotic crossover, wherein one part of the chromosome is duplicated and the other is deleted as it happens in at least part of the PV clone.

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5
Q

JAK2 V617F is located at what chromosome

A

Chromosome 9p

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6
Q

The V617F mutation is present in:

PV:
ET and PMF:

A

PV: All
ET and PMF: more than 50%

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7
Q

In PV (unlike in ET), it is often in its associated (homozygous or heterozygous) form as a result of UPD, at least in some of the progenitors

A

In PV (unlike in ET), it is often in its associated HOMOZYGOUS form as a result of UPD, at least in some of the progenitors

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8
Q

Patients bearing homozygous JAK2 V617F tend to have:

A

Longer duration of disease
Higher hemoglobin levels
Increased incidence of pruritus
More likely to transform to post-polycythemia vera MF (post-PV MF)
Increased spleen volume
Increased leukocytosis
Increased severity of MF

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9
Q

Kind of JAK2 mutation is present in PV patients who are JAK2 V617F -negative

A

JAK2 exon 12 mutations

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10
Q

Patients with JAK2 exon 12 mutations have:

A

Erythrocytosis only
Occurs at a younger age
Higher hemoglobin
Normal EPO level
Differences in marrow morphology- megakaryocytic clusters are largely absent and lack of panmyelosis

Disease course and clinical outcome, however, are similar.

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11
Q

Mutation in patients with polycythemia with eosinophilia which has no evidence of uniparental disomy and all erythropoietin-independent BFU-E were heterozygous for this novel JAK2 mutant.

A

JAK2 exon 13

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12
Q

The risk of acquiring a JAK2 V617F -positive MPN is three- to fourfold higher in patients with the_________________haplotype.

A

JAK2 GGCC (46/1) haplotype

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13
Q

Could be considered be the “fourth” driver mutation in Philadelphia chromosome negative MPNs, in addition to JAK2, CALR and cMPL

A

LNK protein mutations (encoded by SH2B3 gene)

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14
Q

A history of any autoimmune disorder has been associated with a _____ increased risk of developing an MPN

A

20%

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15
Q

PV usually has an insidious onset, most commonly during the _________ decade of life, although onset may occur from childhood to old age.

A

Sixth decade of life

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16
Q

TRUE OR FALSE

Other PV patients may be uncovered during investigation for arterial or venous thrombosis, or iron deficiency.

A

TRUE

Other PV patients may be uncovered during investigation for arterial or venous thrombosis, or iron deficiency.

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17
Q

The most common symptoms of PV

A

Headache

In decreasing order of frequency: headache, fatigue, weakness, pruritus, dizziness, and night sweats, but these symptoms are more likely in patients with PV transforming to MF

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18
Q

The most common and most important complication of PV

A

Thrombotic episodes

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19
Q

Thrombotic episodes occur in about _________ of PV patients

A

One-third

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20
Q

The most common serious complication of PV

A

Cerebrovascular accident

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21
Q

Bleeding and bruising is a common complication of PV, occurring in approximately __________of patients in some series.

A

One-quarter

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22
Q

TRUE OR FALSE

Bleeding is more common in persons with marked thrombocytopenia

A

FALSE

Bleeding is more common in persons with marked thrombocytosis (>1000 × 10 9 /L).

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23
Q

Syndrome caused by a thrombosis in the hepatic venous outflow, leading to ischemia from reduced perfusion through hepatic arterioles and necrosis of hepatocytes

A

Budd-Chiari syndrome

Budd-Chiari syndrome may be the first clinical manifestation of PV

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24
Q

TRUE OR FALSE

PV is the most frequent underlying disease associated with Budd-Chiari syndrome.

A

TRUE

PV is the most frequent underlying disease associated with Budd-Chiari syndrome.

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25
Q

Treatment for Budd-Chiari syndrome

A

Transjugular intrahepatic portosystemic shunt placement Liver transplantation

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26
Q

Cause of aquagenic pruritus in PV patients

A

Increased numbers of mast cells in the skin and elevated histamine levels

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27
Q

Syndrome characterized by warmth of the extremities; painful, reddened digits; a burning sensation; and erythema of the fingers, hands, and feet that is associated with thrombocytosis.

A

Erythromelalgia

  • A causative role for transient microvascular occlusion by platelet aggregates has been proposed
  • It is not specific to PV or other MPNs
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28
Q

Treatment for erythromelalgia

A

Low-dose aspirin

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29
Q

TRUE OR FALSE

It is advised to normalize blood counts and blood volume before surgical interventions, and use specific preventive measures preoperatively.

A

TRUE

It is advised to normalize blood counts and blood volume before surgical interventions, and use specific preventive measures preoperatively.

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30
Q

Phase of PV characterized by a combination of anemia (non–iron deficiency), progressive increase of splenic size, and in most, but not all instances, represents post-PV MF

A

Spent phase of PV

  • First noticed when phlebotomy requirements decrease
  • The appearance of significant anisocytosis and poikilocytosis and teardrop cells (dacryocytes) heralds the onset of the spent phase and postPV MF
  • Associated with an increased risk of leukemic transformation
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31
Q

In the updated 2016 WHO diagnostic criteria for PV, the hemoglobin level must be:

Male:
Female:

A

Male:16.5 g/dL
Female: 16.0 g/dL

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32
Q

TRUE OR FALSE

In patients treated with HU, MCV decreases to very low values

A

FALSE

In patients treated with HU, MCV increases to very high values

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33
Q

TRUE OR FALSE

Red cell mass determination is not useful in distinguishing PV from secondary erythrocytosis because the red cell mass is increased in both disorders.

A

TRUE

Red cell mass determination is not useful in distinguishing PV from secondary erythrocytosis because the red cell mass is increased in both disorders.

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34
Q

The principal value of red cell mass determination is to

A

Distinguish PV from apparent or spurious erythrocytosis
Distinguishing cases of masked PV from ET

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35
Q

Absolute neutrophilia occurs in about __________ of patients with PV.

A

Two-thirds

Neutrophils may be an important factor in PV-associated thrombosis.

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36
Q

Basophilia occurs in approximately _____________of patients with uncontrolled disease.

A

Two-thirds

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37
Q

The platelet count is increased in approximately_______ of patients with PV at the time of diagnosis, and in approximately ______of patients it is greater than 1000 × 10 9 /L

A

50% has increased platelet count

10% of patients it is greater than 1000 × 10 9 /L

38
Q

TRUE OR FALSE

There are no consistent abnormalities of thrombopoietin levels in patients with PV.

A

TRUE

There are no consistent abnormalities of thrombopoietin levels in patients with PV.

39
Q

TRUE OR FALSE

Patients with MPNs often display a nearly pathognomonic defect in the primary wave of platelet aggregation induced by epinephrine

A

TRUE

Patients with MPNs often display a nearly pathognomonic defect in the primary wave of platelet aggregation induced by epinephrine

40
Q

Platelet counts over 1000 × 10 9 /L are associated with

A

Acquired, type 2 von Willebrand disease

Increased risk of bleeding, but not of thrombosis

41
Q

Plasma levels of Vitamin B12 and Uric Acid are (increased or decreased)

A

INCREASED

42
Q

TRUE OR FALSE

Plasma has been used for detection of the JAK2 V617F DNA and mRNA mutation and zygosity state, plasma analysis is reliable.

A

FALSE

Plasma has been used for detection of the JAK2 V617F DNA and mRNA mutation and zygosity state, but plasma analysis is not reliable.

43
Q

TRUE OR FALSE

JAK2 exon 12 mutations are found in ET.

A

FALSE

JAK2 exon 12 mutations are not found in ET.

44
Q

Serum erythropoietin levels in patients with PV is (below or above) the normal reference range.

A

Below the normal reference range

45
Q

TRUE OR FALSE

An elevated erythropoietin level generally excludes the diagnosis of PV, but a normal or even increased erythropoietin levels can be seen in Budd-Chiari syndrome; normal erythropoietin is more likely to be encountered in PV subjects with JAK2 exon 12 mutations.

A

TRUE

An elevated erythropoietin level generally excludes the diagnosis of PV, but a normal or even increased erythropoietin levels can be seen in Budd-Chiari syndrome; normal erythropoietin is more likely to be encountered in PV subjects with JAK2 exon 12 mutations.

46
Q

The most specific and sesitive test for PV

A

Detection of EECs in cultures of blood or marrow

47
Q

In terms of EECs, difference between PV vs PFCP and congenital disorders of hypoxia sensing

A

EECs in PFCP and congenital disorders of hypoxia sensing, are abrogated by pretreatment with erythropoietin and erythropoietin receptor-blocking antibodies

48
Q

TRUE OR FALSE

Absent or decreased iron stores are seen in the marrow of many patients with PV.

A

TRUE

Absent or decreased iron stores are seen in the marrow of many patients with PV.

49
Q

World Health Organization Criteria for
The Diagnosis of Polycythemia Vera, 2016

A

Major Criteria
1. Hemoglobin >16.5 g/dL (men), >16.0 g/dL (women) OR Hematocrit >49% (men), >48% (women) OR Increased red cell mass >25% above mean predicted value
2. Bone marrow biopsy showing hypercellular for age, trilineage myeloproliferation (panmyelosis) including prominent erythroid, granulocytic, and megakaryocytic proliferation with pleomorphic mature megakaryocytes (differences in size)
3. Presence of JAK2V617F or JAK2 exon 12 mutation

Minor Criterion
1. Subnormal serum erythropoietin level

A diagnosis of PV requires the presence of all three major criteria or
the first two major criteria and the one minor criterion.

50
Q

Criterion number 2 (bone marrow biopsy) may not be required in:

A

Sustained absolute erythrocytosis: hemoglobin
levels >18.5 g/dL in men (hematocrit 55.5%) or >16.5 g/dL in women (hematocrit 49.5%)

If major criterion 3 and the minor criterion are
present.

51
Q

The major causes of morbidity and mortality in PV

A
  1. Increased incidence of vascular complications (ie, thrombosis and/or hemorrhage)
  2. Progression to post-PV MF or acute leukemia/ myelodysplasia
52
Q

Major risk factors for serious vascular complications in PV

A

Age of the patient (>60 years)

Previous thrombotic events

53
Q

Classifications of PV

A

Low risk
Intermediate risk: age older than 60 years
High risk: previous thrombotic events (including transient ischemic attacks)

54
Q

The most adverse additional somatic PV mutations in terms of overall, leukemia‐free, and fibrosis‐free survival in PV:

A

ASXL1, SRSF2, and IDH2 mutations

55
Q

Durable response must last at least _____ weeks

A

12 weeks

56
Q

Large improvement in symptoms means:

A

A ≥10-point decrease in MPN
Symptom Assessment Form total assessment score

57
Q

Treatment for plethoric phase

A

Nonspecific myelosuppression
Phlebotomies

+ Aspirin

58
Q

Most commonly used myelosuppressive agents

A

HU and pegylated IFN-α preparations

59
Q

Indicated for those who are resistant or intolerant to HU

A

Targeted JAK2 inhibitors

60
Q

Among treatment for spent phase this have been proven to be beneficial in prospective trials

A

JAK2 inhibitors

61
Q

Treatment for spent phase PV

A

HU
Transfusion
Erythropoiesis-stimulating drugs
JAK2 inhibitors
Hypomethylating agents
Splenectomy
Allogeneic stem cell transplantation

62
Q

Most common myelosuppressive agent used in the treatment of PV

A

Hydroxyurea

initial therapy (1000–1500 mg qd) and long-term treatment (500–2000 mg qd)

63
Q

An effective therapy for controlling erythrocyte, leukocyte, and platelet counts, and it decreases the risk of thrombosis

A

Hydroxyurea

64
Q

About _____ of PV patients develop HU resistance or intolerance (ie, skin ulcers or gastrointestinal intolerance)

A

10%

Resistance to HU has been correlated with decreased survival and a higher rate of transformation to AML or MF.

65
Q

A side effect in a small number of patients with PV, especially in areas with increased UV exposure

A

Nonmelanoma skin cancers

66
Q

Causes long-lasting marrow suppression; can be given intermittently at a dose of 2–8 mg daily for a period not exceeding several weeks

A

Busulfan

67
Q

Has increased incidence of transformation to leukemia

A

Busulfan

68
Q

Dose of Radioactive Phosphorus used in PV

A

2–4 mCi

69
Q

It could be a treatment option for older patients and patients who may be difficult to follow living in remote areas.

A

Radioactive Phosphorus

70
Q

Its use results in a decrease in JAK2V617F allelic burden and conversion from clonal to polyclonal hematopoiesis

A

IFN-α

71
Q

Drug of choice in pregnant PV patients

A

PEG-IFN-α

Better tolerated than standard IFN-α and requires less frequent administration

72
Q

The initial treatment for patients with uncomplicated PV

A

Phlebotomy

73
Q

Recommended therapy for low-risk PV cases:

A

Phlebotomy + low-dose aspirin

74
Q

When phlebotomy is instituted, the hemoglobin may be reduced to normal or near-normal values by the removal of ______ mL of blood at one time every ______days

A

450 mL of blood at one time every 2–4 days

Smaller amounts should be removed from patients who weigh less than 50 kg

75
Q

TRUE OR FALSE

Patients with impaired cardiovascular function are better treated with smaller phlebotomies at less frequent intervals.

A

FALSE

Patients with impaired cardiovascular function are better treated with smaller phlebotomies at more frequent intervals.

76
Q

This treatment does not reduce the leukocyte or platelet count, nor does it affect pruritus or gout.

A

Phlebotomy

77
Q

The usual consequences of repeated phlebotomies:

A

Iron deficiency and resulting microcytosis

78
Q

TRUE OR FALSE

In patients with Chuvash erythrocytosis, the risk of stroke is independent of hematocrit and increased by phlebotomies

A

TRUE

In patients with Chuvash erythrocytosis, the risk of stroke is independent of hematocrit and increased by phlebotomies

79
Q

Can be used in PV to decrease thrombocytosis, as an adjunct to other treatments

A

Anagrelide

80
Q

Starting dose of Anagrelide

A

0.5 or 1.0 mg given four times daily

Average dose required to control the platelet count was 2.4 mg per day

81
Q

TRUE OR FALSE

Control of pruritus is achieved using myelosuppression.

A

TRUE

Control of pruritus is achieved using myelosuppression.

82
Q

Aside from myelosuppression, agents helpful for pruritus

A

Photochemotherapy with psoralens and ultraviolet light

83
Q

The study that showed that daily low-dose aspirin decreased arterial and venous thromboses, albeit incompletely.

A

ECLAP study

84
Q

TRUE OR FALSE

Aspirin should be used when the platelet count exceeds 1000–1500 × 109/L

A

FALSE

Aspirin should not be used when the platelet count exceeds 1000–1500 × 109/L

85
Q

An oral JAK1/JAK2 inhibitor is now FDA-approved for patients with PV that is resistant or intolerant to HU.

A

Ruxolitinib

86
Q

TRUE OR FALSE

JAK2 inhibition does not change or modify the natural course of PV, but mainly ameliorates the symptoms.

A

TRUE

JAK2 inhibition does not change or modify the natural course of PV, but mainly ameliorates the symptoms.

No conclusive evidence of its benefits for prevention of thromboses and transformation to post-PV MF and AML.

87
Q

An HDAC inhibitor that specifically inhibits the proliferation of JAK2V617F-positive cells compared with normal cells by inhibiting hematopoietic transcription factors NFE2 and c-MYB.

A

Givinostat

88
Q

Post-PV MF usually occurs after_______ years or more

A

15 years

89
Q

The treatment of choice in patients with early signs of MDS/AML transformation, it is the only treatment offering the possibility of a cure

A

Hematopoietic Stem Cell Transplantation

90
Q

TRUE OR FALSE

Chlorambucil should no longer be used for PV therapy.

A

TRUE

WILLIAM'S HEMATOLOGY (109 decks)

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