Essential Thrombocythemia with Dr. Raajit Rampal

In this episode, we discuss the diagnosis and management of Essential Thrombocythemia with Dr. Raajit Rampal. Here are the shownotes:
1. IPSET (revised) system, risk categories are defined by the presence of a prior thrombosis, age, and JAK2 mutation status:
https://ashpublications.org/blood/art...

2. The MIPSS-ET provides points for:
· https://onlinelibrary.wiley.com/doi/a...

3. Aspirin
o Is there a benefit of twice-daily aspirin dosing, especially in high-risk or JAK2-mutant disease?
https://ashpublications.org/blood/art...

4. Cytoreduction in High-risk ET

Hydroxyurea: https://www.nejm.org/doi/full/10.1056...

Pegylated interferon alfa

MPD-RC 112 Mascarenhas J et al. A randomized phase 3 trial of interferon- α vs hydroxyurea in polycythemia vera and essential thrombocythemia. Blood . 2022)
https://ashpublications.org/blood/art...

Anagrelide in ET
https://ashpublications.org/blood/art...

5. Ruxolitinib
In a randomized study phase 2 study (MAJIC-ET), ruxolitinib treatment did not deliver better rates of hematological response than best-available therapy, although symptoms did improve.

· MAJIC-ET study which is a randomized phase 2 trial
· ET patients resistant/intolerant to HU were randomized to receive RUX or standard treatment (HU 71%, anagrelide 48%, IFN 40%). The primary study outcome was CR, defined by normal platelet and white cell counts and normal spleen size.
· At 1 year, response was achieved in 46% of patients in the RUX group and in 44% in the standard arm, without statistically significant difference between the two groups.
· In addition, no difference was observed in the rates of thrombosis, hemorrhage, and disease transformation after 2 years of follow-up.
· MRs were also uncommon. RUX was superior, however, in symptom control.
https://ashpublications.org/blood/art...