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Hematology > Hematology Week 3: Myeloproliferative Disorders > Flashcards

Hematology Week 3: Myeloproliferative Disorders Flashcards

1
Q

Myeloproliferative Neoplasm Definition

A
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2
Q

Myeloproliferative Disorders leads to

A
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3
Q

What drives myeloproliferative neoplasm growth?

A

It is unregulated growth, NOT cytokine-mediated

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4
Q

Key types of MPN

3 listed

A

Polycythemia Vera (PV)

Essential Thrombocythemia (ET)

Primary myelofibrosis (PMF)

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5
Q

Polycythemia Vera (PV) Key features

A
  • Overproduction of RBCs

keep in mind MPNs are stem cell disorders so platelets are increased as well but RBC are the prominent form overproduced

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6
Q

Essential Thrombocythemia (ET) Key Features

A

Prominent overproduction of platelets

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7
Q

Primary Myelofibrosis (PMF) Key Features

2 listed

A
  • An initial proliferative picture with gradual BM fibrosis
  • often see leukocytosis and thrombocytosis early on
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8
Q

Key features of MPNs Overview

A
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9
Q

Pathogenesis of MPNs

2 listed

A
  • Tyrosine Kinase Mutations resulting in constitutive activity
  • Usually in JAK2 (others are MPL and CALR)
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10
Q

JAK2 Point Mutation

A

JAK2 will be phosphorylated without EPO

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11
Q

MPL Receptor mutation

A

MPL constitutively activated without TPO

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12
Q

CALR Mutation

A

CALR is a highly conserved protein with pleiotropic roles related to its distribution in the endosplasmic reticulum and cytosol, and on the cell surface. In cellular assays, transfection of mutant CALR leads to hyperactivation of the JAK-STAT pathway

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13
Q

JAK2 Mutations in MPN

A

An acquired point mutation in JAK2 (9p) results in a constitutive cytoplasmic tyrosine kinase activity

  • growth factor independence
  • other proliferative/survival advantages
  • V617F phenylalanine substituted for valine
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14
Q

JAK2 Mutation Amino Acid change

A

V617F phenylalanine substituted for valine

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15
Q

Acute Myeloid Leukemia Cell types

A
  • Blasts predominate
  • Maturation impaired/blocked
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16
Q

Chronic Myeloid Leukemia (CML) Cell types

A
  • Neutrophils and other mature cells predominate
  • Type of Myeloproliferative neoplasm
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17
Q

Polycythemia Vera Cell types

A
  • Mature RBCs, platelets or leukocytes predominate
  • myeloproliferative neoplasm
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18
Q

Essential Thrombocythemia (ET) Cell Types

A
  • Mature RBCs, platelets or leukocytes predominate
  • myeloproliferative neoplasm
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19
Q

Myelodysplasia Cell Types

A
  • Mature Cells predominate but are reduced in number in the blood
  • Cytopenia(s)
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20
Q

MDS/MPN/AML Blood Smears

A

MDS - cytopenias

MPN - Cytosis

AML - can be cytopenias, cytosis, or normal BUT ALWAYS Neutropenia, severe anemia, severe thrombocytopenia

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21
Q

MDS/CML/AML BM Features

A

MDS

CML

AML - blasts everywhere

MPN - mature cells

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22
Q

AML Blood Findings

A
  • Profound cytopenias
  • Variable numbers of circulating blasts
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23
Q

Myelodysplasia Blood Findings

A

Cytopenias

always at least one sustained cytopenia (anemia, neutropenia, or thrombocytopenia)

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24
Q

Myeloproliferative neoplasms Blood Findings

A

Elevated cell counts (erythrocytosis, thrombocytosis, leukocytosis (neutrophils, other mature WBCs predominate) (Sustained)

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25
Q

1st genetically defined leukemia

26
Q

Always check for?

A

`BCR-ABL1 for cytosis before JAK2 to rule out CML because great targeted therapy that works

27
Q

MPNs diagnostic criteria

A

genetic features are key

28
Q

MPNs disorders are characterized by

A
  • uncontrolled cell proliferation (usually multiple lineages)
  • with intact maturation
29
Q

MPN common hepatosplenomegaly

30
Q

MPNs cheat growth regulation

A

growth factor independent neoplasms

31
Q

Most common type of MPN

A

Polycythemia Vera (PV)

32
Q

Polycythemia Vera (PV) Pathogenesis

A

JAK2 mutation leading to constitutive Tyrosine Kinase activity

33
Q

Polycythemia Vera (PV) Hallmarkl

A

excess production of mature RBCs but platelets are often increased as well

34
Q

Polycythemia Vera (PV) Disease course

A

typically indolent but thrombosis or bleeding are significant risk factors

35
Q

Polycythemia Vera (PV) may progress to?

A

BM Fibrosis

36
Q

Polycythemia Vera (PV) skin changes

A

Polycythemia rubra vera skin changes

37
Q

Polycythemia Vera (PV) Blood smear

A

Hgb should of been 13

Hct should be 42-44

Plt should of been ~150,000

38
Q

Polycythemia Vera (PV) BM

A

her bone marrow is very cellular

older patients should have lots of fat

these are signs of myeloproliferative neoplasm

39
Q

Other causes of erythrocytosis

3 listed

A
  • Chronic hypoxia - Cardiopulmonary disease is EPO-mediated
  • EPO-producing neoplasms - Renal cell carcinoma or other tumors that are EPO-mediated
  • Reduced plasma volume - Hemoconcentration from fluid loss (emesis, dehydration) usually transient NOT EPO-Mediated
40
Q

Essential Thrombocythemia (ET) Hallmark

A
  • High platelet count
  • Thrombocytosis is sustained and is not related to any acute traumatic event
  • Platelet count might exceed one million/uL (1,000 on CBC)
41
Q

Essential Thrombocythemia (ET) BM findings

A

Megakaryocytes are markedly increased

42
Q

Essential Thrombocythemia (ET) Pathogenesis

A

result of JAK2 or (CALR or MPL) mutation resulting in constitutive Tyrosine Kinase activity

43
Q

Essential Thrombocythemia (ET) Disease course

A

indolent but risk of thrombosis (or rarely bleeding) are significant issues

44
Q

BM of Essential Thrombocythemia (ET)

A

BM of Essential Thrombocythemia (ET)

lots of megakaryocytes and thrombocytosis

45
Q

Primary Myelofibrosis (PMF) Hallmark

A

begin with leukocytosis, possible thrombocytosis and nucleated RBCs

gradually progressive BM failure

gradual progressing splenomegaly

46
Q

Primary Myelofibrosis (PMF) physical manifestation

A

can cause Massive Splenomegaly

47
Q

Primary Myelofibrosis (PMF) BM

A

Fibrosis in BM

48
Q

Myeloproliferative Neoplasms overview

A

too much of a good thing

49
Q

Excess RBC production can cause

3 listed

A
  • Plethora/splenomegaly
  • Sludging
  • Thrombosis/hemorrhage
50
Q

Excess platelet production

2 listed

A
  • thrombosis/hemorrhage
  • spontaneous abortions due to thrombosis
51
Q

Excess WBC and platelets production with BM firbrosis can cause

2 listed

A
  • Splenomegaly
  • Thrombosis
52
Q

Question 1

53
Q

Treatment of PV

7 listed

A
  • The goal is to reduce the risk of thrombosis (both venous and arterial)
  • rarely can become CML
  • can draw blood (phlebotomy)
  • Cytoreductive therapy hydroxyurea
  • interferon
  • JAK2 inhibitors show promise
  • Low dose aspirin
54
Q

Treatment of ET

4 listed

55
Q

PV high-risk patients

2 listed

A
  • >60 yo
  • history of thrombosis
56
Q

ET high-risk patients

2 listed

A
  • >60 yo
  • history of thrombosis
57
Q

Treatment of PMF Anemia

6 listed

A
  • Variable due to variable presentation/symptoms
  • Often begin with watchful waiting approach
  • only curative option is HSCT
  • Anemia
  • EPO stimulators
  • Immunomodulatory agents (thalidomide/lenalidomide)
58
Q

Treatment of PMF Splenomegaly

A

JAK2 inhibitors seem to be really helpful in shrinking spleen size

thrombocytopenia is the dose-limiting factor for JAK2 inhibitors

59
Q

Treatment of PMF pharmacology

A
  • EPO can cause thrombosis so have to weigh risk and benefits in a patient with a JAK2 mutation
  • GCSF can prevent overly neutropenia
60
Q

Risk Stratification of PMF

A

IPSS - International Prognostic Scoring System

Hematology (26 decks)

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