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Mercy CLS 6: Hematology > 20) MPN > Flashcards

20) MPN Flashcards

1
Q

hypercellularity of the BM
dominance of one cell line in PB

A

MPN

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2
Q

3 most common cell lines involved in MPNs

A
  • myeloid (CML)
  • megakaryocytic (ET)
  • erythroid (PV)
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3
Q

less common cell lines involved in MPNs

A
  • fibroblasts
  • eosinophils
  • mast cells
  • variable
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4
Q

MPN age group

A

40s-60s

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5
Q

in MPN, the BM eventually becomes ———

A

fibrotic

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6
Q

MPN can develop into…

A

acute leukemia

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7
Q

why might MPN result in splenomegaly?

A

EMH after BM fibrosis

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8
Q

only MPN with Philadelphia chromosome present

A

CML

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9
Q

3 stages of CML

A
  • chronic: 2-10 years, responds well to therapy
  • accelerated: 6-18 months
  • blast crisis: 3-6 months, aggressive acute leukemia
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10
Q

median CML age of onset

A

70-90 years

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11
Q

loss of stamina
weakness
night sweats
weight loss
feeling of fullness
hepatosplenomegaly

A

CML

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12
Q

first signs of CML might be…

A

GI bleeding
retinal hemorrhage

plts don’t function well

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13
Q

clonal disorder of HSC; overproduction of defective granulocytes

A

CML

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14
Q

Philadelphia chromosome cell of origin

A

HSC

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15
Q

the Philadelphia chromosome is a juxtaposition of ——– proto-oncogene with a portion of ——- gene

A

ABL1
BCR

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16
Q

the novel BCR/ABL1 gene produces…

A

p210 fusion protein with abnormal tyrosine kinase activity

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17
Q

tyrosine kinase inhibitors

A

used to tx CML

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18
Q

platelets in CML

A

often > 1000 x 10^3

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19
Q

M:E 10-50:1

A

CML

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20
Q

leukemic hiatus is NOT seen in…

A

CML

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21
Q

CML erythropoiesis is normally…

A

normoblastic

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22
Q

may see pseudo-Gaucher
cells overloaded with cerebrosides

A

CML

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23
Q

indications of accelarated phase CML

A
  • 10-19% blasts in PB
  • basos in PB
  • persistent thrombocytopenia or thrombocytosis
  • additional chromosomal abnormalities
  • 2+ mutations in BCR/ABL1 during TKI therapy (TKI resistance)
  • splenomegaly unresponsive to therapy
24
Q

blast crisis is ——- phase CML

A

terminal
clinical features similar to acute leukemia

25
Q

CML can result in (AML/ALL) blast crisis

A

either one

mostly AML, but some ALL with Ph chromosome

26
Q

rare MPN with sustained PB neutrophilia (>25 x 10^3) and mild left shift

A

chronic neutrophilic leukemia (CNL)

27
Q

CNL is often associated with this cancer

28
Q

CNL median survival

29
Q

parts of CNL diagnosis of exclusion

A
  • other causes of neutrophilia ruled out
  • no Ph chromosome
  • no PDGFRA, PDGFRB, or FGFR1 mutations
  • CSF3R mutation may be present
30
Q

CSF3R mutation

31
Q

bands + segs > 80% WBC
no dysgranulopoiesis

32
Q

ET

A

essential thrombocythemia

MPN

33
Q

pulmonary embolism
h/a
gangrene of toes and fingers
hemorrhagic episodes
palpable spleen

34
Q

ET median survival

A

19.8 years

35
Q

ET can transform into…

A

PMF
acute leukemia

36
Q

extreme and consistent thrombocytosis
giant bizarre platelets

37
Q

anemia in ET, if present, is ——-/——-

A

normo/normo

38
Q

mutations in JAK2, MPL, or CALR

39
Q

mutation JAK2V617 rules out…

40
Q

PMF

A

primary myelofibrosis

41
Q

——— is a very common cause of polycythemia

(not PV)

42
Q

unregulated proliferation of erythroid elements in BM, and ↑ RBC

43
Q

mutations common in PV

A

JAK2 (V617F)
JAK2 exon 12

44
Q

must differentiate PV from…

A
  • relative polycythemia (↓ plasma volume)
  • secondary polycythemia (↑ EPO)
45
Q

inappropriate ↑ EPO causing polycythemia may be caused by…

A

renal tumor
smoking
high altitude
renal ischemia

46
Q

PV
↑ RBC mass → ————– → CV disease

A

blood hyperviscosity

47
Q

1/3 of PV patients have ——– or ——— episodes

A

thrombotic
hemorrhagic

48
Q

Hct >60%

49
Q

PV factors affecting survival

A
  • age
  • abnormal karyotype
  • leukocytosis
  • history of thrombosis
50
Q

Hgb & Hct diagnostic requirements for PV (male and female)

A

Male
Hct: >49%
Hgb: >16.5

Female
Hct: >48%
Hgb: >16.0

51
Q

PV often shows —–lineage growth in BM

52
Q

EPO will be —– in PV

53
Q

nonspecific RBC morphology associated with PV

A

poik
targets
pencil cells
acanthocytes

54
Q

most aggressive MPN

A

primary myelofibrosis

55
Q

PMF survival

56
Q

mutations common in PMF

A

JAK2
CALR
MPL

57
Q

PB findings in PMF

A

teardrops
blasts
large plts

Mercy CLS 6: Hematology (25 decks)

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