Blood Cancer Talks

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)
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
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.

What is Blood Cancer Talks?

This is a podcast on latest advances in the understanding and management of blood cancers. Here, we will bring a wide range of experts within hematologic malignancies to discuss various topics in depth.
Host: Raj Chakraborty, MD from Columbia University, New York, Ashwin Kishtagari, MD, from Vanderbilt University, Nashville, and Edward Cliff, MD, from Harvard University, Boston
Tweet your suggestions and feedback to @rajshekharucms @AshKishtagari @Eddie_Cliff @BloodCancerTalk