More than 200 cancer patients, supporters, and physicians/researchers gathered at Mayo Clinic Scottsdale this morning at 8 am to begin a lively conference about the state of knowledge and treatment of the Myeloproliferative Neoplasms — Essential Thrombocythemia, Polycythemia Vera, and Myelo Fibrosis.
Dr. Ruben Mesa opened the session and welcomed everyone and reminds us that this conference is because of Joyce Niblack. Her husband Bob carries on her work through the CMPD Education Foundation with this Biennial Conference.
It will take some time to prepare proper notes from the conference, as the presenters had visual presentations and talked quickly. When I get all the information together, I will post a complete written report.
Here are a few nuggets from today:
The hematocrit that feels best for you is where you should be. While some people feel good at 42 or 45, others may need it lower to feel good, like 38 – 40. Don’t let your doctor get stuck on a prescribed number if it doesn’t feel good for you.
Don’t worry about tracking your JAK2 allele burden over time; we don’t know that it has any clinical relevance. All the drugs today inhibit the JAK2.
JAK2 inhibitors are unlikely to eliminate the disease.
Whole exome sequencing to identifyMPN alleles and gene sequencing is becoming more affordable.
Soon there will be a lot of data; they will be able to learn to identify what genes cause the transformation to leukemia.
In your genes:
2 copies of JAK2 => PV
1 copy of JAK2 => ET
Future in drug therapies: combine drugs in rational combinations to facilitate next level of clinical trials
Prolonged use of interferon can reverse fibrosis in the bone marrow (does not cure the disease).
Help for Pruritis (itchy skin):
- JAK2 inhibitors are good.
- Interferon is better than HU.
- UV narrow band B works 70% of time (see dermatologist)
- Weight wraps (prednisone in a wrap) (see dermatologist)
- Aprepitant, an anti-emetic, works on the Substance P
Formication: the sensation of ants crawling on your body.
Hematopoietic Cell Transplantation (HCT):
The only treatment that has potential for a cure for MPNs is the stem cell transplant.
Pegasys (Pegylated Interferon) is more potent than regular Interferon-a. The dose is not so important as the duration. The effect is probably due to your own biologic abilities.
Standard practice regarding Phlebotomies: no more than 4 per year.
If you are going to get a splenectomy, go to one of the half dozen surgeons who have done the procedure on MPN patients. Removing the spleen has real risks: clots in the area; 5-6% mortality (similar to open heart surgery).
When to have another Bone Marrow Biopsy (BMB)? only if concerned the diseased has progressed to Myelofibrosis.
That’s all for now!