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Hematology 2 > Myeloproliferative Neoplasms (MPNs) > Flashcards

Myeloproliferative Neoplasms (MPNs) Flashcards

1
Q

MPNs

A

monoclonal hyperprolifferation abnormalities (neoplasms) or myeloproliferative neoplasms.

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2
Q

What are the four types of MPNs?

A
  • PV
  • CML
  • ET
  • PMF (primary myelofibrosis)
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3
Q

Polycythemia Vera

A

unregulated proliferation of BM erythroid, granulocytic & megakaryocytes

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4
Q

PV Etiology

A
  • gene mutation JAK2
    -even though hyperactivity of JAK2 may not initiate PV, it is associated
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5
Q

JAK2 gene

A

nonreceptor tyrosine kinase that plays a role in EPO and TPO receptors

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6
Q

PV diagnosis

A

-2 major and 1 minor criteria must be met.

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7
Q

2 major criteria

A
  • Hgb 18.5 M; 16.5F
  • ID of JAK2 mutation
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8
Q

1 minor criteria

A
  • panmyelosis in BM
  • low EPO
  • autonomous, erythroid
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9
Q

PV clinical symptoms

A
  • Ruddy face
  • itchy skin
  • HTN
  • vertigo
  • feeling full
    -blurred VA
    -↑uric acid
    -splenomegaly
    -↑B12 serum
  • ↓Fe storage or absent
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10
Q

Lab findings

A
  • RBCt=6-10x10^6/uL- severe crowding
    -difficult pB smear
  • Hgb>18
  • Hct=55-60%
    -ESR =0-3mm/hr
    -n or ↑WBC & plts w/ n. morph
    -immature granulocytes
  • plts=400,000 to 2 mil
    -BM hypercellular w/worsening fibrosis
  • ↓FE stable to spent phase
    -nRBC
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11
Q

PV Tx’t

A

no cure, therapeutic phlebotomy 1st choice, myelosupressive drugs: ↓BV & ↑Fe stores.

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12
Q

Explain the etiology of Chromic Myelocytic Leukemia

A

an extreme elevation of mature/immature myeloid cells in BM which then show up in the p.B.
-This arises from a single translocation putting c-ABL next to BCR yielding BCR/ABL.
-BCR/ABL=↑ tyrosine kinase activity leading to hyperproliferation + unstable.

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13
Q

What are the lab findings in CML

A
  • “when pB looks like B.M.”
  • remember extreme elevation so >100,000/uL & usually 200,000-500,000/uL w/ neutrophilia, basophilia, & eosinophilia
  • p.B has huge shift to the left w/ all developmental stages
  • > 20% basophilia comes before blast crisis
  • Abnormal cytogenetics
  • M:E ratio 10:1-50:1 but when blasts are <30%
  • ↑ plts or ↓ (>700,000 or <150,000/uL
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14
Q

What are the diagnostic testing for CML?

A
  • karyotyping (chromosomal)
    -FISH (cellular level)
  • PCR (molecular level)
    -LAP decreased in early stages
  • BM shows striking increase in M:E ratio ranging from 10:1 to 50:1
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15
Q

Differentiate between the three phases of CML

A
  • Chronic
  • Accelerated: poor response to therapies and increased symptoms
  • Blastic: 3/4 of pts undergo “blast transformation”. Blast >20% which can then indicate turning into either AML or ALL (rare).
    *blastic phase involves pB, BM, & extramedullary tissues
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16
Q

What is treatment for CML & under what circumstances?

A
  • primarily tx’t involved BMT for pts in chronic stage.
    -Now Gleevec (imatinib mesylate) which binds to BCR/ABL causes a decrease in # GF receptors. This leads to abn. cell to die and increase in normal cell proliferation.
  • molecular remission observed
    -“cures” 88% in chronic
17
Q

What is the etiology of Primary Myelofibrosis (PMF)?

A

hematopoetic stem cell disorder of JAK2 in 65% of pts leading to hyperproliferation of BM fibrosis. Fibrosis occurs in response to neoplastic mutation elswhere causing an increase of GF. Can convert to AML

18
Q

What are the clinical symptoms of PMF?

A
  • SEVERE hepatosplenomegaly
  • abdominal pain
  • normo normo due to myeopthisic anemia
19
Q

What are the lab findings in PMF?

A
  • striking pB w/ aniso/poik w/ teardrops/dacrocytes
    -poik becomes more severe as dz progresses to BM failure
  • left shift & ↑WBC count 15-30,000 /uL
  • DRY TAP due to bone marrow fibrosis
    -fibrosis does not allow proliferation of cells
20
Q

What is the treatment of PMF?

A

-chemo
-steroids
-splenectomy
- eventual BMT
-pts usually die from 2 complications: overwhelming infections or massive hemorrhage.

21
Q

What is the etiology of Essential thrombocytothemia?

A

HSC disorder with extreme increased megakaryopoiesis
-plt count ↑>600,000/uL
-plt fxn is abnormal unless plateletpheresis performed to reduce plt ct leaving plt crowding

22
Q

What are clinical symptoms of ET?

A
  • abdominal pain, epistaxis due to abnormal plts fxn, GI bleeding, & bleeding after minor dental sx
23
Q

What are lab findings in ET?

A

600,000-2.5mil/uL
-giant / bizarre plts
-no nRBC
- slt left shift
- no megK fragments

24
Q

What are the difference between ET and M7?

A

ET has +giant plts, noNRBC, slt left shift, no megK fragments.

M7 has giant plts, +nRBC, rare left shift, +megK fragm.

25
Q

What are ET treatments?

A

Myelosuppressive drugs
-IFN-α if myelosuppressive drubs cannot be given
- plt apheresis to reduce plt mass

26
Q

What peripheral blood abnormalities does CNL have?

A

elevated neutrophil count 80%>

27
Q

What BM abn does CNL have?

A

hypercellular, dysplasia and fibrosis usually not present

28
Q

What possible genetic abnormalities does CNL have?

A
  • CSF3RT618I or other activating CSF3R
29
Q

What pB abn does CEL have?

A

WBC>30%

30
Q

What BM abd does CEL have?

A

-fibrosis is common, increased eosinophils

Hematology 2 (6 decks)

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