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Hematology Module 2 > Myeloproliferative Neoplasms > Flashcards

Myeloproliferative Neoplasms Flashcards

0
Q

MPNs are predominantly ________ , not acute

A

chronic

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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

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2
Q

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

A

CML

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3
Q

disease with overproduction of erythrocytes

A

PV

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4
Q

Disease with increased megakaryopoiesis and thrombocytosis

A

ET

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5
Q

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

A

PMF

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6
Q

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

A

CML Chronic Myelogenous Leukemia

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7
Q

Which one is the derivative chromosome in t(9;22) in CML?

A

22

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8
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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9
Q

Laboratory findings in CML PB

A

MK’d leukocytosis, bimodal granulocyte pattern (hallmark), basophilia (Eos can be higher too), Rare nRBC, +/- micromegakaryocytes, increased LD & Uric acid, decreased LAP (stain)

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10
Q

this enzyme is found within secondary granules of neutrophils

A

Leukocyte ALKALINE phosphatase (LAP)

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11
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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12
Q

What is a normal LAP score?

A

Normal 20-100

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13
Q

Is the LAP increased or decreased in untreated CML?

A

decreased

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14
Q

Is the LAP increased or decreased in a leukemoid reaction?

A

normal to increased

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15
Q

Is the LAP increased or decreased in Pregnancy and Polycythemia Vera (PV)?

A

Increased

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16
Q

What do lab findings look like in CML Bone Marrow?

A

Marked hypercellularity, decreased normoblasts (decreased RBC production), normal to increased megakaryocytes, pseudo-gaucher cells often, and increased reticulin fibrosis

17
Q

Most patients are diagnosed in the ______ phase of CML, where blasts are <_____%

A

chronic; 10%

18
Q

The ______ phase of CML has a poorer response to therapy, increased anemia, increased clinical symptoms, micromegakaryocytes, etc

A

Accelerated

19
Q

Blast crisis is classified as >____% Blasts; worst prognosis

A

20%

20
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 or lymphoid

21
Q

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

A

NEG; POS

22
Q

How is CML treated?

A

Synthetic tyrosine kinase inhibitors such as Imatinib (Gleevec), BM transplant, chemo to reduce WBC

24
Q

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

A

Imatinib

25
Q

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

A

CNL has an increased LAP (not decreased like CML)

26
Q

What is Polycythemia Vera’s mutation?

A

JAK2 V617F

27
Q

PV has in increase in what?

A

RBCs/neutrophils/platelets

28
Q

Main clinical presentation of PV

A

Pruritus (but also CNS probs, vision, heart occlusions, ruddy complexion, ulcers, weight loss, splenomegaly)

29
Q

PV bone marrow manifestations

A

hypocellular, but M:E is normal
BM iron store are decreased
Megakaryocytes are increased

30
Q

What is the serum EPO level in PV?

A

below the reference range

31
Q

Three stages of PV

A

Prodromal: mild erythrocytosis
Overt: significant increased red cell mass
Spent: pancytopenia, progresive splenmegaly, BM fibrosis and progression to acute leukemia in 15% of patients

32
Q

Therapy for PV?

A

Phlebotomy

33
Q

ET female to male ratio?

A

2:1

34
Q

5 criteria for diagnosing ET?

A
  • platelet count >450
  • prolif of megakaryocytic line
  • meet criteria for MPN,MDS or Myeloid neoplasm
  • Positive for JAK2
  • Presence of BM iron (help dif from PV)
35
Q

Clinical presentation of ET

A
  • erythromyalgia
  • seizures
  • MI
  • dizziness
  • Platelet dysfunction
36
Q

ET treatment

A

combination of hydroxyurea and low dose aspirin

37
Q

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

A

Primary Myelofibrosis (PMF)

38
Q

Three progressive stages of PMF

A

Prefibrotic Stage: no significant increase in BM fibrosis
Early Stage:
Fibrotic Stage: significant increase in fibrosis

39
Q

PML lab presentation

A

Leukoerythroblastosis

  • tear drops
  • NRBC
  • Polychromasia
  • Some macrocytosis
  • Micromegakaryocytes
40
Q

PML Bone Marrow

A

Dry tap due to fibrosis

41
Q

PML sometimes transforms into what?

A

acute leukemia especially acute myelomonocytic leukemia

Hematology Module 2 (14 decks)

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