Myeloproliferative Neoplasms (MPNs)

Question 1

What are MPNs?

Answer 1

clonal disorder of hematopoietic stem cell caused by genetic mutation, excess of cells in BM

Question 2

T/F: MPNs cause increased positive feedback

Answer 2

false, they cause decreased negative feedback

Question 3

Are MPNs chronic or acute? What kind of maturation?

Answer 3

Chronic w normal maturation

Question 4

What are some MPN disease examples?

Answer 4

CML, CNL, CMoL, PV, ET, PMF, unclassifiable

Question 5

What are a few characteristics of MPNS?
Hint: P/E/O/I/P

Answer 5

panhyperplasia of BM (more than one cell line)
Extramedullay hematopoiesis
overlapping dieases
increased megakaryocytes
PLT dysfunction (cryogenic abnormal)

Question 6

Describe characteristics of CML, ages, important factors (hint Ph) Tyrosine kinase activity?

Answer 6

Chronic myeloid leukemia
mostly pt 45yrs or older
Philadelphia Chrom (9,22)
increased TK activity (uncontrolled proliferation of myeloid cells)

Question 7

T/F: In CML apoptosis is still working

Answer 7

False it does not work

Question 8

What are some lab findings of CML?
WBC counts
how many Pt are Ph22 positive? what does negative mean?
BM?
LAP?
shift to the left?

Answer 8

WBC very high (blood resembles marrow) Increased Basos
90% ph22 pos, negative is atypical disease and less responsive
BM is hyper cellular M:E 10-50:1
LAP decreased
shift to the left with <10% blasts

Question 9

What is the CML accelerated phase? (CML-AP)

Answer 9

10-19% blasts
>20% basos in blood
high abc unresponsive
progresses to acute leukemia
additional clonal cytogenetic abnormality (ICC)

Question 10

what drugs inhibits the tyrosine kinase activity in CML (also BM)?

Answer 10

Glecvec (this is prob not important tbh)

Question 11

Describe CNL, common? WBC counts, M:E ratio, Shift to the left, LAP? What about inclusions?

Answer 11

Chronic neutrophilic leukemia
VERY rare, 1.8yr survival
M:E ratio 20:1
no shift to the left
WBC >80% bands/neuts
LAP Very HIGH
Dohle bodies/toxic gran

Question 12

Describe CMoL/CMML

Answer 12

Chronic monocytic leukemia
now is CMML
is a MDS/MPN

Question 13

Describe Polycythemia Vera (Rubra Vera)
RBC
Mutations
EPO?
HCT/HGB

Answer 13

Increased RBC MASS
JAK2 gene mutation
Hgb/Hct increase
EPO decreased
RBC production w/out EPO

Question 14

What does the BM look like in PV?
LAP?
O2 saturation?
Iron deficiency?

Answer 14

BM is hyper cellular
LAP increased
O2 saturation normal
iron deficiency develops, bleeding/increased rbc production and Fe turnover

Question 15

T/F: There is no issue w sodium citrate tubes and PV

Answer 15

false there is issues, with the anticoagulant to plasma ratios (too much anticoagulant)

Question 16

What are some long term PV things that happen?

Answer 16

tear drop cells, myeloid fibrosis,
hearlds most common transition of PV to spent phase

Question 17

Describe secondary polycythemia

Answer 17

not malignant, due to hypoxia
decreased O2
EPO increased
normal gran/PLT
LAP normal

Question 18

describe relative erythrocytosis
most common groups

Answer 18

normal O2, EPO/Gran/PLT
Low plasma
HCT increased
normal rbc mass
Common groups:
Dehydration
or middle aged white male w smoking problems and overweight

Question 19

Describe Essential Thrombocythemia (ET)
plt level
mutation

Answer 19

increased megakary-poesis
VERY high PLT
spontaneous PLT aggregation - thrombotic hemorrhage
JAK2 mutation

Question 20

Describe PMF
BM
how does it present?
mutations?
taps?
WBC?
RBC morph?

Answer 20

primary myelofibrosis can present denovo or progression of PV or ET
marrow fibrosis (collagen)
JAK2 Mutation
BM drytap
pancytopenia
tear drop rbcs
LOW WBC/PLTS