ET facts

Question 1

ACAs % in ET

Answer 1

50%

Question 2

Most common ACAs in ET

Answer 2

TET2 10%
ASXL1 15%
DNMT3A 7%
SF3B1 5%

Question 3

Abnormal karyotype in ET %

Answer 3

> 10%
+9
del 20q
del 13q

Question 4

Mutations with incresased risk of post ET-PMF

Answer 4

CALR1
MPL

Question 5

mutation specific phenotype in ET

Answer 5

CALR, TN- thrombocytosis (type 2 more than type 1)
JAK2- leukocytosis, higher Hb

Question 6

risk factots for leukemic transformation in ET

Answer 6

Pre-fibrotic PMF
Thrombosis
Extrema thrombocytosis in JAK2 positive
Abnormal karyotype

Question 7

risks for post-ET PMF

Answer 7

Pre-fibrotic PMF
CALR, MPL
Advanced age
Anemia
Leukocytosis for JAK2 positive

Question 8

Risk scores on ET

Answer 8

IPSET
MIPSS-ET
AAA

Question 9

IPSET

Answer 9

age>60
WBC>11
Thrombosis Hx

Question 10

MIPSS-ET

Answer 10

age>60
male
WBC>11
adverse mutations-
SF3B1, SRSF2, U2AF1, P53

Question 10

AAA in ET

Answer 10

Age> 70, 2 points, 50-70 1 point
ALC<1.7
ANC>8

Question 11

benefit of bid aspirin in ET

Answer 11

laboratory studies showed better tromboxan inhibition
possible beenfit in extreme thrombocytosis and int risk ET

Question 12

2nd line Tx for high risk ET

Answer 12

peg-inf for pts under 65
busulfan for pts above 65

Question 13

Fetal loss in ET %

Answer 13

30-40%