MDS Flashcards
What are some low risk MDS genetic defining events?
MDS w/5q deletion
MDS w/SF3B1 mutation
These are both low blast/low risk MDS
What is a high risk MDS genetic defining event?
MDS with biallelic TP53 mutations.
What are some high risk cytogenetic abnormalities with MDS?
Think anything with chromosome 3 is bad. Inv3, del3q, t(3q), del7
What are some good risk MDS cytogenetic abnormalities? If you don’t remember all of these it’s okay, more so important to know the high risk ones.
del 5q, del 11q, del 12p, del 20q
What MDS mutation is associated with a good prognosis and is the only one for this?
SF3B1 mutation
When treating a MDS patient who has anemia with a ESA agent, what level predicts for response?
If they have a level of 200 or less they most likely will get a response, otherwise it likely won’t work.
What is Luspatercept approved for in MDS?
It is approved for the treatment of very low risk to intermediate risk MDS w/ringed sideroblasts that is refractory to a ESA or will not respond to ESA due to high EPO. Also approved for MDS/MPN-RS-T.
When using Luspatercept in MDS what signaling is affected and how?
It decreases SMAD2 and SMAD3 signaling (normally when this pathway is active it inhibits RBC maturation).
What is the approval for Lenalidomide for MDS?
Patients with low risk to intermediate risk disease with 5q del who are transfusion dependent
What is the mechanism of action of Lenalidomide in MDS?
It modulates E3 ubiqutin ligase cereblon activity, this in turn alters the degradation of casein kinase 1 which increases Wnt/B-catenin signaling
What is the only medication that is associated with a survival benefit in high risk MDS besides transplant?
Azacitidine
Remember to yourself, what defines high risk MDS, in terms of blast count, platelet, and Hgb?
Marrow Blast Count>10%
Hgb less than 8
Platelet Count less than 50K
What are the treatment options for high risk MDS who are not eligible for transplant?
Azacitidine, Decitabine, Oral decitabine in combination with cedazuridine
All high risk patients with MDS should be pushed for what?
Transplant unless there are contraindications.
