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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
CML can result in (AML/ALL) blast crisis
either one mostly AML, but some ALL with Ph chromosome
26
rare MPN with sustained PB neutrophilia (>25 x 10^3) and mild left shift
chronic neutrophilic leukemia (CNL)
27
CNL is often associated with this cancer
MM
28
CNL median survival
2.5 years
29
parts of CNL diagnosis of exclusion
- other causes of neutrophilia ruled out - no Ph chromosome - no PDGFRA, PDGFRB, or FGFR1 mutations - CSF3R mutation may be present
30
CSF3R mutation
CNL
31
bands + segs > 80% WBC no dysgranulopoiesis
CNL
32
ET
essential thrombocythemia | MPN
33
pulmonary embolism h/a gangrene of toes and fingers hemorrhagic episodes palpable spleen
ET
34
ET median survival
19.8 years
35
ET can transform into...
PMF acute leukemia
36
extreme and consistent thrombocytosis giant bizarre platelets
ET
37
anemia in ET, if present, is -------/-------
normo/normo
38
mutations in JAK2, MPL, or CALR
ET
39
mutation JAK2V617 rules out...
CML
40
PMF
primary myelofibrosis
41
--------- is a very common cause of polycythemia (not PV)
smoking
42
unregulated proliferation of erythroid elements in BM, and ↑ RBC
PV
43
mutations common in PV
JAK2 (V617F) JAK2 exon 12
44
must differentiate PV from...
- relative polycythemia (↓ plasma volume) - secondary polycythemia (↑ EPO)
45
inappropriate ↑ EPO causing polycythemia may be caused by...
renal tumor smoking high altitude renal ischemia
46
PV ↑ RBC mass → -------------- → CV disease
blood hyperviscosity
47
1/3 of PV patients have -------- or --------- episodes
thrombotic hemorrhagic
48
Hct >60%
PV
49
PV factors affecting survival
- age - abnormal karyotype - leukocytosis - history of thrombosis
50
Hgb & Hct diagnostic requirements for PV (male and female)
**Male** Hct: >49% Hgb: >16.5 **Female** Hct: >48% Hgb: >16.0
51
PV often shows -----lineage growth in BM
tri
52
EPO will be ----- in PV
↓↓
53
nonspecific RBC morphology associated with PV
poik targets pencil cells acanthocytes
54
most aggressive MPN
primary myelofibrosis
55
PMF survival
4-5 years
56
mutations common in PMF
JAK2 CALR MPL
57
PB findings in PMF
teardrops blasts large plts

Mercy CLS 6: Hematology (25 decks)

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