ET

Question 1

M:F

Answer 1

1:2

Question 2

Median age

Answer 2

60

Question 3

Yong patients

Answer 3

< 40 years

20%

Question 4

Clinical manifstations

Answer 4

Splenomegaly 35%
Vasomotor symp 20%
Thrombosis 20%
Bleeding 10%

Question 5

Predictors of arterial thrombosis

Answer 5

Age > 60
JAK2
CV risk factors
WBC > 11K

Question 6

Predictors of venous thrombosis

Answer 6

Male gender

Question 7

Predictors for bleeding

Answer 7

PLT > 1M
Aspirin or NSIADS
AC

Question 8

Pregnancy complications

Answer 8

1st trimester abortions 45%
IUGR
Preeclampsia

does not corelate with disease course. Aspirin may reduce risk.

Question 9

BM fidings

Answer 9

Large to giant megakaryocytes with:
Abundant mature cytoplasm
Deeply lobulated
Hyperlobulated nuclei

Question 10

BM fidings

Answer 10

Large to giant megakaryocytes with:
Abundant mature cytoplasm
Deeply lobulated
Hyperlobulated nuclei

Question 11

Driver mutations %

Answer 11

JAK2– 60-65%
CALR– 20-25%
MPL– 5%
Triple negative – 10-15%

Question 12

Genetic mutations clinical manifstations

Answer 12

JAK2- high HB and WBC, low PLT, thrombosis, progression
CALR- high PLT. low HB and WBC, less thrombosis, same progression
MPL- lacks prognostic meaning

Question 13

WHO diagnostic criteria

Answer 13

PLT > 450K
BM showing typical findings
CML, PMF and PV excluded
JAK2/CALR/MPL

Question 14

Pretreatemnt avaluation

Answer 14

Thrombosis history
Vasomotor symptoms
Splenomegaly
Genetic mutations
if PLT> 1M or bleeding- VWF activity
Calculate- IPSET-thrombosis score

Question 15

IPSET-thrombosis score

Answer 15

age > 60
JAK2
Thrombosis history

CV risk factors also important but not part of score

Question 16

Tx of low risk

Answer 16

Aspirin

Question 17

Tx of intermediate and high risk

Answer 17

Aspirin +

Cytoreductive agent

Question 18

Cytoreductive agents

Answer 18

HU
Anagrelide
Pegylated interferon

Question 19

Twice daily aspirin indactions

Answer 19

Vasomotor symp despite once daily aspirin

Pts with JAK2 and CV risk factors

Question 20

Anagrelide mechanism of action

Answer 20

Inhibits cyclic nucleotide phosphodiesterase

Question 21

Hydrooxyurea side effects

Answer 21

Leukopenia
Cutaneous vasculitis
ILD
Hemolytic anemia
Oral ulcers
Nail changes
Hyperpigmentation

Question 22

Anagrelide side effects

Answer 22

Cardoimyopathy

Question 23

Anagrelide side effects

Answer 23

Cardoimyopathy

Question 24

Pegylated interferon alfa-2a

Answer 24

Not leukomgenic
2nd line after HU
safe in pregnancy

Question 25

Indications for platletpharesis

Answer 25

Extreme PLT count with:

Bleeding/Thrombosis