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Episode 43. Essential Thrombocythemia with Dr. Raajit Rampal
Manage episode 413202707 series 3369804
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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:
· Very low risk: No prior thrombosis, age ≤60 years, JAK2-unmutated
· Low risk: No prior thrombosis, age ≤60 years, JAK2-mutated
· Intermediate risk: No thrombosis, age >60 years, JAK2-unmutated
· High risk: History of thrombosis (any age/genotype), or age >60 years with JAK2 mutation
2. The MIPSS-ET provides points for:
· Age > 60 years (3 points)
· Adverse mutation (SF3B1/SRSF2/U2AF1/TP53) (2 points)
· Male sex (1 point)
· White blood cell count ≥11 × 10^9/L (1 point)
· https://onlinelibrary.wiley.com/doi/abs/10.1111/bjh.16380?sid=nlm%3Apubmed
3. Aspirin
o Is there a benefit of twice-daily aspirin dosing, especially in high-risk or JAK2-mutant disease?
4. Cytoreduction in High-risk ET
Hydroxyurea: https://www.nejm.org/doi/full/10.1056/NEJM199504273321704
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)
Anagrelide in ET
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.
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