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Episode 43. Essential Thrombocythemia with Dr. Raajit Rampal
Manage episode 413202707 series 3369804
Innehåll tillhandahållet av Rajshekhar Chakraborty and Ashwin Kishtagari, Rajshekhar Chakraborty, Ashwin Kishtagari, and Edward Cliff. Allt poddinnehåll inklusive avsnitt, grafik och podcastbeskrivningar laddas upp och tillhandahålls direkt av Rajshekhar Chakraborty and Ashwin Kishtagari, Rajshekhar Chakraborty, Ashwin Kishtagari, and Edward Cliff eller deras podcastplattformspartner. Om du tror att någon använder ditt upphovsrättsskyddade verk utan din tillåtelse kan du följa processen som beskrivs här https://sv.player.fm/legal.
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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