ET facts

Question 1

ACAs % in ET

Answer 1

50%

Question 2

Mutation specific phenotype in ET

Answer 2

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

Question 3

Risk factots for leukemic transformation in ET

Answer 3

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

SRSF2, SF3B1, U2AF1, p53
Age
Leukocytosis
Male

Question 4

Risks for post-ET PMF

Answer 4

Pre-fibrotic PMF
CALR type 1, MPL
Advanced age
Anemia
Leukocytosis for JAK2 positive

Question 5

Risk scores in ET

Answer 5

IPSET
MIPSS-ET
AAA

Question 6

IPSET- thrombosis

Answer 6

Age>60
Prior VTE
CV risk factors
JAK2

Question 7

MIPSS-ET

Answer 7

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

Question 7

Benefit of bid aspirin in ET

Answer 7

Laboratory studies showed better tromboxan inhibition
Possible benefit in extreme thrombocytosis and int risk ET

Question 8

2nd line Tx for high risk ET

Answer 8

peg-inf for pts < 65
Busulfan for pts > 65

Question 9

Fetal loss in ET %

Answer 9

30-40%