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Boards.CP.Heme > MPNs: CML, PV, ET, PMF, CEL > Flashcards

MPNs: CML, PV, ET, PMF, CEL Flashcards

1
Q

Which MPN has the longest survival and lowest transformation (5%) to Acute Leukemia?

A

Essential Thrombocythemia (ET)

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2
Q

What is the Diagnostic Criteria for ET? (4)

Aimed at excluding secondary thrombocytosis and other MPNs

A
  • Sustained Thrombocytosis (>450)
  • BM w/ Megakaryocytic Hyperplasia w/O panmyelosis (no significant increase in granulocytic/erythroid precursors)
  • Fails to meet criteria for PV, PMF, CML, or MDS
  • JAK2 B617F mutation (or no evidence for reactive thromobocytosis)
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3
Q

CML Imatinib Resistance:

-% Initially present

A

-5% of cases initially (emerges during treatment in many more)

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4
Q

Philadelphia Chromosome:

-Most commonly occurs where and with what?

A

In major breakpoint cluster (M-BCR)

-w/ p210 fusion protein

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5
Q 
Essential Thrombocythemia (ET):
-Bone Marrow findings (4)
A
  • Large, Hyperlobated Megs
  • Paratrabecular
  • Emperipolesis
  • Stainable Iron Present
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6
Q

Most common cause of death in PV?

-2nd

A

Thrombosis

-Acute Leukemia

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7
Q

Mutations in what, produce a small subset of ET and PMF but are NOT seen in PV?

A

MPL mutations

-MPL W515L or W515K

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8
Q

What is the Philadelphia Chromosome?

A

Reciprocal Translocation of chromosomes 9 and 22

-t(9;22)(q34;q11)

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9
Q 
Primary Myelofibrosis (PMF) - Cellular (Prefibrotic) Stage:
-Presentation (3)
A
  • Anemia
  • Mild Leukocytosis
  • Thrombocytosis
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10
Q 
Polycythemia Vera (PV) - Diagnostic Criteria:
-General (2)
A
  • Both Major + 1 Minor

- 1st Major + 2 Minor

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11
Q

What is the Jak-2 protein involved in?

A

Stimulating the STAT pathway

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12
Q

CML Imatinib Resistance:

-Result of what?

A

Mutations within the BCR-ABL gene

  • Tyrosine Kinase Domain
  • P-loop (so-called)
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13
Q

CML is defined by the presence of what?

A

Philadelphia Chromosome

-t(9;22) - BCR/ABL

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14
Q

Essential Thrombocythemia (ET):

  • Incidence
  • Presentation
A
  • Bimodal (peaks at age 30 and 60)

- Isolated Thrombocytosis

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15
Q

CML - Blast Phase manifests as what types of acute leukemia? (2)
-Percentages

A
  • AML (70%)

- ALL (30%)

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16
Q

CML - Chronic Phase:

-Peripheral Blood (6)

A 
Leukocytosis with:
-Neutrophilia
-Monocytosis
-Basophilia
-Eosinophilia
Thrombocytosis
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17
Q

What is the most powerful Prognostic Factor in CML?

A

Response to Tyrosine Kinase Inhibitor (TKI) therapy as measured by Quantitative RT-PCR.

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18
Q

Chronic Eosinophilic Leukemia (CEL):

-Must exclude other hematolymphoid neoplasms w/ eosinophilia (3)

A

Rearrangements of:

  • PDGFRa
  • PDGFRb
  • FGFR1
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19
Q

CML - Accelerated Phase criteria:

-1 or more of the following (4)

A
  • Progressive Basophilia (>20%)
  • Progressive Thrombocytopenia (<100) or Thrombocytosis (>1,000)
  • Clonal Cytogenetic Progression (+8,i17q,+19,2ndPhChr)
  • Increasing Blasts (>10% but <20%)
20
Q 
Polycythemia Vera (PV) - Proliferative (Chronic) Phase:
-Peripheral Blood (4)
A

Erythrocytosis - sometimes w/:

-Neutrophilia, Basophilia, and/or Thrombocytosis

21
Q

Which uncommon breakpoint/fusion protein is associated with marked thrombocytosis and relatively mature leukemic neutrophils?

A

u-BCR breakpoint

-p230 fusion protein

22
Q

Which uncommon breakpoint/fusion protein is associated with CML with marked monocytosis and Ph+ ALL?

A

m-BCR breakpoint

-p190 fusion protein

23
Q

What is the most common JAK-2 Mutation?

A

G to T substitution at nucleotide 1849

-Val to Phe substitution at codon 617 (Val617Phe)

24
Q

CML - Chronic Phase:

-Bone Marrow (5)

A
  • Hypercellular BM
  • High M:E ratio
  • Small,Hypolobated (“Dwarf”) Megs
  • Mild Reticulin Fibrosis
  • Thickening of Paratrabecular generative cuffs
25
Q

Chronic Eosinophilic Leukemia (CEL):

-Common tissue sites (4)

A
  • Heart
  • GI tract
  • Lung
  • CNS
26
Q

Chronic Eosinophilic Leukemia (CEL) must have evidence of clonality, such as increased blasts or clonal cytogenetic abnormalities; lack this and the diagnosis is?

A

Hypereosinophilic Syndrome (HES)

27
Q

Primary Myelofibrosis (PMF) - Cellular (Prefibrotic) Stage:
-Bone Marrow finding (1)
-Megakaryocyte morphology/chromatin/distribution
-

A

-Hypercellular BM

Megakaryocytes:

  • Lobulated (aberrantly)
  • Clumped, Hyperchromatic, and Inky Chromatin
  • Clusters adjacent to sinuses and trabeculae
28
Q

CML - Chronic Phase:

  • Splenomegaly (+/-)
  • Leukocyte Alkaline Phosphatase (LAP) score
  • Vit. B12 level
A

CML - Chronic Phase:

  • Splenomegaly is Common
  • LOW Leukocyte Alkaline Phosphatase (LAP) score
  • Elevated Vit. B12 level (markedly)
29
Q 
Polycythemia Vera (PV) - Diagnostic Criteria:
-Minor Criteria (3)
A
  • BM panmyelosis (megs,grans,erythroids)
  • Endogenous Erythroid Colony Formation (in vitro)
  • Normal serum EPO
30
Q

Chronic Eosinophilic Leukemia (CEL):

-Must exclude secondary eosinophila causes (4)

A
  • Allergic reaction
  • Parasitic infection
  • Collagen Vascular Disease
  • Mastocytosis
31
Q 
Polycythemia Vera (PV) - Diagnostic Criteria:
-Major Criteria (2)
A

First Major:

  • Hgb >18.5 (Men)
  • Hgb >16.5 (Female)
  • or Increased RBC Mass

Second Major:

  • JAK2 B617F
  • or functionally similar JAK2 mutation
32
Q 
Polycythemia Vera (PV):
-Signs/Symptoms (7)
A
  • HTN
  • Thrombosis
  • Pruritus
  • Plethora
  • Erythromelalgia
  • Headache
  • Splenomegaly
33
Q

CML - Blast Phase criteria. (3)

A
  • > 20% Blasts in Blood or BM
  • Tissue infiltrate of Blasts (Chloroma)
  • Accumulation of Blasts in BM filling an Intertrabecular Space)
34
Q

Chronic Eosinophilic Leukemia (CEL):

-Diagnostic Criteria

A

Eosinophilia (>1.5) with tissue infiltration and damage

35
Q

What mutation is present in the majority of non-CML MPNs?

-Percentages

A

JAK2 (Janus Kinase 2) Mutation

  • PV (>90%)
  • ET/PMF (>50%)
36
Q 
Primary Myelofibrosis (PMF) - Fibrotic Stage:
-Peripheral blood (1)
A

Leukoerythroblastic pattern

37
Q 
Primary Myelofibrosis (PMF) - Fibrotic Stage:
-Bone Marrow (4)
A
  • Bone Marrow is Inaspirable
  • Reticulin Fibrosis
  • Intrasinusoidal Hematopoiesis
  • Abnormal, Clustered Megs
38
Q

Chronic Eosinophilic Leukemia (CEL):

  • Gender
  • Age
A
  • M>F (9:1)

- 25-45 y/o

39
Q

What are the Myeloproliferative Neoplasms? (5)

A 
-Chronic Myelogenous Leukemia (CML)
Non-CML MPNs
-Polycythemia Vera (PV)
-Essential Thrombocythemia (ET)
-Primary Myelofibrosis (PMF)
-Chronic Eosinophilic Leukemia (CEL)
40
Q 
Polycythemia Vera (PV) - Spent Phase:
-AKA
A

Postpolycythemic Myelofibrosis with Myeloid Metaplasia (PPMM)

41
Q 
Polycythemia Vera (PV) - Spent Phase:
-Findings (3)
A
  • Peripheral Myelophthisic Pattern
  • BM Reticulin Fibrosis
  • Extramedullary Hematopoiesis
42
Q

Chronic Eosinophilic Leukemia (CEL):

-Eosinophil characteristics (2)

A
  • Hypogranular

- Cyanide Resistant MPO stain highlights granules

43
Q

A second activating mutation involving what, produces a small proportion of cases?

A

JAK2 exon 12

44
Q 
Polycythemia Vera (PV) - Proliferative (Chronic) Phase:
-Bone Marrow (3)
A
  • Hypercellular BM
  • Megakaryocyte Hyperplasia (Prominent)
  • Decreased/Absent Stainable Iron
45
Q

Philadelphia Chromosome:

  • ABL locus
  • BCR locus
  • Result
A
  • ABL locus - 9q34
  • BCR locus - 22q11
  • Chimeric Bcr-Abl protein with enhanced Tyrosine Kinase Activity
46
Q

CML - Chronic Phase:

-Neutrophils

A
  • Immature Neutrophils

- High proportion of Myelocytes (Myelocyte “Bulge”)

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