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

Myeloproliferative Neoplasms (MPNs) Flashcards

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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
Patients who are Philadelphia Chrom _______ have a worse prognosis than Ph _______ because it progresses more rapidly
Negative; positive
26
How is CML treated?
- Synthetic tyrosine kinase inhibitors such as Imatinib (Gleevec), - BM transplant (autologous or allogenic) - Chemo or alpha-interferon to reduce WBC
27
This drug competes for the binding site of the BCR-ABL gene and reduces activity of the tyrosine kinase
Imatinib
28
Chronic Neutrophilic Leukemia is not CML but its close. What is a big difference between the two?
- CNL has an ↑ LAP | - CML has a ↓ LAP
29
What is Polycythemia Vera's mutation?
JAK2 V617F
30
Polycythemia Vera PB findings
Increase in RBCs, neutrophils, platelets
31
Main clinical presentation of PV
- 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
Main clinical presentation of CML
- Easy fatigability - Abdominal discomfort - Weight loss - Night sweats - Splenomegaly - Bone pain/tenderness - Gout
33
Polycythemia Vera BM findings
- Hypercellular (but M/E ratio is usually normal) - ↓ BM iron stores - ↑ megakaryocytes - Panmyelosis?
34
Polycythemia Vera | - Major diagnostic critera (2)
- Hgb > 18.5 g/dL in men; > 16.5 g/dL in women | - Presence of JAK2 V617F or other functionally similar mutation
35
Polycythemia Vera | - Minor diagnostic criteria (3)
- Hypercellularity in BM for all lineages - Serum EPO level below reference range - Endogenous erythroid colony formation in vitro
36
Three stages of Polycythemia Vera
- Prodromal - Overt - Spent
37
Polycythemia vera | - Therapy
Phlebotomy
38
Polycythemia Vera | - Prodomal (stable) phase
Borderline to mild erythrocytosis
39
Polycythemia vera | - Overt phase
Significant increase in red cell mass
40
Polycythemia Vera | - Spent phase
- Pancytopenia - Progressive splenomegaly - BM fibrosis - Progression to acute leukemia in 15% of patients
41
Polycythemia Vera | - Pathogenesis
- Pluripotential stem cell clone - Decline in normal CFU-GM - Increase in CFU-GEMM
42
What is EPO more hypersensitive in PV?
Even the smallest amount of EPO will activate RBCs to divide/grow/mature
43
Polycythemia Vera | - Other helpful findings in diagnosis
- ↑ 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
Polycythemia Vera prognosis
- Untreated: 6-18 months | - Treated: 14-18 years
45
- Secondary cause of erythrocytosis in PV | - Relative cause of erythrocytosis in PV
- Secondary: ↑ RBC (or hemoglobin) as a result of ↓ O2 | - Relative: apparent increase in RBCs or hgb due to a decrease in plasma volume
46
ET female to male ratio?
2:1
47
What 4 criteria must be met for diagnosing ET?
- 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
Other helpful criteria for diagnosis of ET
- Hgb < 13.0 x 10^9/L - Normal erythrocyte mass; presence of BM iron - No leukoerythroblastic reaction - No known cause of reactive thrombocytosis
49
How to differentiate ET from PV
There is presence of BM iron in ET
50
How to differentiate ET from CML
There is an absence of the Philadelphia C'some in ET
51
How to differentiate ET from PMF
There is an absence of marrow collagen fibrosis in ET
52
Clinical presentation of ET
- 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
Essential Thrombocythemia PB findings
- 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
Essential Thrombocythemia BM findings
- megakaryocyte hypercellularity (↑ megakaryocyte diameter w/ nuclear hyperlobulation) - Loose clustering of meagkaryocyte - ↑ erythropoiesis only if hemorrhaging has occurred - Normal granulopoiesis
55
Treatment for Essential Thrombocythemia
Combination of hydroxyurea and low dose aspirin to prevent hemorrhagic and vaso-occlusive complications
56
Characterized by proliferation of megakaryocytic and granulocytes. At a later stages there is excessive bone marrow fibrosis
Primary Myelofibrosis (PMF)
57
Other characteristics of PMF
- Myeloid metaplasia (spleen, liver, LNs) = extramedullary hematopoiesis - Progressive hepato/splenomegaly - Leukoerythroblastosis - Myelodysplasia
58
Three progressive stages of PMF
- Prefibrotic stage - Early stage - Fibrotic stage
59
What happens in the prefibrotic stage of PMF?
- 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
What happens in the fibrotic stage of PMF?
- 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
PMF lab presentation
- 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
PMF BM presentation
Dry tap to fibrosis - granulocytic and megakaryocytic hyperplasia - Dysmegakaryopoiesis - Dysgranulopoiesis
63
CML may transform into ____
Acute lymphoblastic leukemia
64
Chronic myelomonocytic leukemia is classified in the WHO system as what?
Myelodysplastic syndrome/myeloproliferative neoplasm (MDS/MPN)

Liz's Hematology Module 2 (13 decks)

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