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Liz's Hematology Module 2 > Myeloproliferative Neoplasms (MPNs) > Flashcards

Myeloproliferative Neoplasms (MPNs) Flashcards

1
Q

Clonal hematopoietic stem cell diseases with expansion, excessive production and over accumulation of erythrocytes, granulocytes and platelets in some combination; usually aquired

A

Myeloproliferative neoplasms (MPNs)

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2
Q

MPNs are predominantly ____, not acute

A

Chronic

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3
Q

Disease with overproduction of granulocytes; the cells do differentiate; clonal disorder of HSC (hematopoietic stem cell line)

A

Chronic Myelogenous Leukemia (CML)

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4
Q

Disease w/ overproduction of erythrocytes

A

Polycythemia Vera (or Polycythemia Ruba Vera) (PV)

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5
Q

Disease w/ increased megakaryopoiesis and thrombocytosis

A

Essential Thrombocythemia (ET)

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6
Q

Combination of overproduction of hematopoietic cells and ineffective hematopoiesis with resultant PB cytopenias; fibrosis in BM

A

PMF

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7
Q

Characterized by acquired structural chromosomal abnormality: Philadelphia chromosome t(9;22)(q34;q11.2), creates the fusion gene BCR-ABL1

A

CML

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8
Q

Which c’some is the derivative c’some in t(9;22) in CML?

A

C’some 22

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9
Q

Unique chimeric gene in CML that codes from a protein with enhanced tyrosine kinase activity which leads to increased proliferation and a loss of apoptotic functions

A

BCR/ABL1

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10
Q

Laboratory findings in CML PB

A
  • Mk’d leukocytosis
  • Bimodal granulocyte pattern (hallmark)
  • Basophila (eosinophils can be higher too)
  • Rare nRBCs
  • +/- micromegakaryocytes
  • ↑ LD and uric acid
  • ↓ LAP (stain)
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11
Q

What enzyme is found w/in secondary granules of neutrophils?

A

Leukocyte ALKALINE phosphatase (LAP)

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12
Q

Philadelphia C’some

A

t(9;22)(q34; q11.2)

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13
Q

CML

- Theory of pathogenesis

A

ABL proto-oncogene from band q34 of c’some 9 is moved to the BCR region of band q11 on c’some 22

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14
Q

How do you score the LAP stain? KNOW HOW TO CALCULATE

A

Score 100 cells from 0-4+ and total the scores; 4+ being a cell that has a lot of secondary granules

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15
Q

What is a normal LAP score?

A

20-100

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16
Q

Is the LAP increased or decreased in untreated CML?

A

Decreased

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17
Q

Is the LAP score increased or decreased in a leukemoid reaction?

A

Normal to increased

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18
Q

Is the LAP increased or decreased in pregnancy and Polycythemia Vera?

A

Increased

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19
Q

What do lab findings look like in CML BM?

A
  • Mk’d hypercellularity
  • ↓ normobloasts (b/c of ↓ RBC production)
  • Normal to ↑ megakaryocytes
  • Pseudo-Gaucher cells (often)
  • ↑ reticulin fibrosis
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20
Q

What are the 3 stages of CML?`

A
  • Chronic or stable
  • Accelerated
  • Blast crisis
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21
Q

What stage of CML?

- < 10% blasts

A

Chronic or stable

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22
Q

What stage of CML?

  • Poorer response to therapy; more severe anemia; increase in clinical symptoms; micromegakaryocytes; additional c’some abnormalities
  • Blasts = 10-19% or
  • Promyelocytes and blasts combined = 20%
A

Accelerated

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23
Q

What stage of CML?

- > 20% blasts; worst prognosis

A

Blast crisis

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24
Q

In CML blast crisis extramedually tissues such as the liver and spleen are involved; since the stem cell is affected, blasts can go either way ______ or ______

A

Myeloid; lymphoid

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25
Q

Patients who are Philadelphia Chrom _______ have a worse prognosis than Ph _______ because it progresses more rapidly

A

Negative; positive

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26
Q

How is CML treated?

A
  • Synthetic tyrosine kinase inhibitors such as Imatinib (Gleevec),
  • BM transplant (autologous or allogenic)
  • Chemo or alpha-interferon to reduce WBC
27
Q

This drug competes for the binding site of the BCR-ABL gene and reduces activity of the tyrosine kinase

A

Imatinib

28
Q

Chronic Neutrophilic Leukemia is not CML but its close. What is a big difference between the two?

A
  • CNL has an ↑ LAP

- CML has a ↓ LAP

29
Q

What is Polycythemia Vera’s mutation?

A

JAK2 V617F

30
Q

Polycythemia Vera PB findings

A

Increase in RBCs, neutrophils, platelets

31
Q

Main clinical presentation of PV

A
  • Pruritus
  • Splenomegaly/hepatomegaly
  • CNS (headache, tinnitus → thicker blood)
  • Blurred vision → thicker blood
  • Arterial/venous occlusions (b/c of ↑ platelets)
  • ↑ hemorrhagic/thrombotic risk (circulatory problems)
  • Dusky/ruddy complexion/plethora
  • Peptic ulcers
  • Weight loss/fatigue
32
Q

Main clinical presentation of CML

A
  • Easy fatigability
  • Abdominal discomfort
  • Weight loss
  • Night sweats
  • Splenomegaly
  • Bone pain/tenderness
  • Gout
33
Q

Polycythemia Vera BM findings

A
  • Hypercellular (but M/E ratio is usually normal)
  • ↓ BM iron stores
  • ↑ megakaryocytes
  • Panmyelosis?
34
Q

Polycythemia Vera

- Major diagnostic critera (2)

A
  • Hgb > 18.5 g/dL in men; > 16.5 g/dL in women

- Presence of JAK2 V617F or other functionally similar mutation

35
Q

Polycythemia Vera

- Minor diagnostic criteria (3)

A
  • Hypercellularity in BM for all lineages
  • Serum EPO level below reference range
  • Endogenous erythroid colony formation in vitro
36
Q

Three stages of Polycythemia Vera

A
  • Prodromal
  • Overt
  • Spent
37
Q

Polycythemia vera

- Therapy

A

Phlebotomy

38
Q

Polycythemia Vera

- Prodomal (stable) phase

A

Borderline to mild erythrocytosis

39
Q

Polycythemia vera

- Overt phase

A

Significant increase in red cell mass

40
Q

Polycythemia Vera

- Spent phase

A
  • Pancytopenia
  • Progressive splenomegaly
  • BM fibrosis
  • Progression to acute leukemia in 15% of patients
41
Q

Polycythemia Vera

- Pathogenesis

A
  • Pluripotential stem cell clone
  • Decline in normal CFU-GM
  • Increase in CFU-GEMM
42
Q

What is EPO more hypersensitive in PV?

A

Even the smallest amount of EPO will activate RBCs to divide/grow/mature

43
Q

Polycythemia Vera

- Other helpful findings in diagnosis

A
  • ↑ RBC mass (≥ 36 mL/kg in males; ≥32 mL/kg in females)
  • O2 saturation ≥92% (normal)
  • Platelets: > 400 x 10^9/L
  • Leukocytosis: > 12 X 10^9/L in absence of infection
  • ↑ LAP, serum vitamin B12 and binding capacity
  • Iron deficiency will lead to M/H presentation
44
Q

Polycythemia Vera prognosis

A
  • Untreated: 6-18 months

- Treated: 14-18 years

45
Q
  • Secondary cause of erythrocytosis in PV

- Relative cause of erythrocytosis in PV

A
  • Secondary: ↑ RBC (or hemoglobin) as a result of ↓ O2

- Relative: apparent increase in RBCs or hgb due to a decrease in plasma volume

46
Q

ET female to male ratio?

A

2:1

47
Q

What 4 criteria must be met for diagnosing ET?

A
  • Sustained platelet count of > 450 x 10^9/L
  • Proliferation of megakaryocytic line in BM w/ ↑ numbers of enlarged, mature megakaryocytes
  • Must NOT meet criteria for other MPN, MDS, or myeloid neoplasm
  • Positive for JAK2 V617F (found in 40-50% of ET cases)
48
Q

Other helpful criteria for diagnosis of ET

A
  • Hgb < 13.0 x 10^9/L
  • Normal erythrocyte mass; presence of BM iron
  • No leukoerythroblastic reaction
  • No known cause of reactive thrombocytosis
49
Q

How to differentiate ET from PV

A

There is presence of BM iron in ET

50
Q

How to differentiate ET from CML

A

There is an absence of the Philadelphia C’some in ET

51
Q

How to differentiate ET from PMF

A

There is an absence of marrow collagen fibrosis in ET

52
Q

Clinical presentation of ET

A
  • Erythromelalgia
  • Seizures
  • Cerebral or myocardial infarction
  • Headache, dizziness, visual disturbances
  • Platelet dysfunction
  • Thrombosis (extremities, CNS, coronary circulation)
  • Hemorrhage (bruising, epistaxis, GI hemorrhage, postoperative hemorrhage)
53
Q

Essential Thrombocythemia PB findings

A
  • Thrombocytosis (giant, agranular, pseudopods)
  • Platelets cluster and accumulate near feather of blood film
  • RBCs N/N
  • Mature neutrophils may be increased (basophils are not)
54
Q

Essential Thrombocythemia BM findings

A
  • megakaryocyte hypercellularity (↑ megakaryocyte diameter w/ nuclear hyperlobulation)
  • Loose clustering of meagkaryocyte
  • ↑ erythropoiesis only if hemorrhaging has occurred
  • Normal granulopoiesis
55
Q

Treatment for Essential Thrombocythemia

A

Combination of hydroxyurea and low dose aspirin to prevent hemorrhagic and vaso-occlusive complications

56
Q

Characterized by proliferation of megakaryocytic and granulocytes. At a later stages there is excessive bone marrow fibrosis

A

Primary Myelofibrosis (PMF)

57
Q

Other characteristics of PMF

A
  • Myeloid metaplasia (spleen, liver, LNs) = extramedullary hematopoiesis
  • Progressive hepato/splenomegaly
  • Leukoerythroblastosis
  • Myelodysplasia
58
Q

Three progressive stages of PMF

A
  • Prefibrotic stage
  • Early stage
  • Fibrotic stage
59
Q

What happens in the prefibrotic stage of PMF?

A
  • No significant increase in BM fibrosis
  • BM is hypercellular w/ increases in neutrophils and megakaryocytes (abnormal forms)
  • Neutrophils may have a left shift but usually metas, bands, and segs predominate
60
Q

What happens in the fibrotic stage of PMF?

A
  • Significant increases in BM fibrosis
  • Holistically the BM is hypocellular but there can be focal hypercellular areas
  • Abnormal megakaryocytes appear in large clusters
  • Acute phase > 20% blasts
61
Q

PMF lab presentation

A
  • Leukoerythroblastosis (tear drops, nRBC, polychromasia, some macrocytosis, micromegakaryocytes from progressive folate deficiency)
  • Granulocytes (immature granulocytes, blasts, dysplastic cells)
  • Increased platelets, micromegakaryocytes, and platelet fragments
  • JAK2 V617F
  • Increased ALP, LDH, uric acid, LAP
62
Q

PMF BM presentation

A

Dry tap to fibrosis

  • granulocytic and megakaryocytic hyperplasia
  • Dysmegakaryopoiesis
  • Dysgranulopoiesis
63
Q

CML may transform into ____

A

Acute lymphoblastic leukemia

64
Q

Chronic myelomonocytic leukemia is classified in the WHO system as what?

A

Myelodysplastic syndrome/myeloproliferative neoplasm (MDS/MPN)

Liz's Hematology Module 2 (13 decks)

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