Polycythemia Vera: What is next when treatment stops working?

How has your mother been treated so far? What medication has she been taking? Perhaps a second opinion?

She was given Jakavi and Hydroxylurea almost from the begining.she has anemia so she takes Jakavi 3times a week. At first the doctor was encouraging that we are at really early and manageable stages.but at biopsy i saw that she had 6-7%blasts which with the white cells remainig high,i think it gives more of an aggressive picture.

I can only respond from my personal experience. I was diagnosed four years ago. Medications will manage Polycytemia Vera so that it is not fatal. I have my blood tested every three months in order to be sure the dosage is correct, (eastern U.S. here) and that blood components are not at dangerous levels. It seems that you need more communication with your doctors for a long term care plan. Anxiety can affect overall heath.

Hi @oliviamitza Welcome to Mayo Connect. I’m so sorry to hear that your mom’s blood condition doesn’t appear to be responding to treatment. That’s disheartening for both of you. From what you’ve mentioned, your mom was diagnosied with Polycythemia Vera two years ago, a condition where her body is producing an excess amount of red blood cells.
Most often this condition can be managed quite well with medications that your mom’s doctor has prescribed; Jakafi or Hydroxyurea. But from my understanding there is a small percentage of patients with PV where the condition may transform to “acute leukemia (blast phase) 10 years after diagnosis in about 10% of cases”. (*articles cited below). Even though your mum was diagnosed only two years ago, PV is often symptomless and found with routine blood work. So she may have developed PV many years ago before being diagnosed.

The presence of blast cells in her tests is an indication that this is now more aggressive. Blast cells are immature blood cells and normally only present in the bone marrow where blood develops. But when they appear in blood tests or at an abnormally high level in a bone marrow biopsy, that can be an indication of leukemia. Left untreated, this doesn’t generally have a positive outcome.

I’m not a medical professional but had my own experience with leukemia several years ago with an 85% blast cell count. This required aggressive chemotherapy and a bone marrow transplant. I didn’t have the same PV diagnosis as your mom but still on the myeloid side of the blood. So I’m wondering, since she has moved to a blast phase of the disease if she could be considered for a less intensity chemotherapy such as Vidaza and/or Venectoclax. These are chemotherapy drugs for older adults who have Acute myeloid leukemia (AML) or Myelodyplastic syndrome (MDS). Quite often these drugs can help control the progression or even reach remission and then continued as a maintenance drug. Again, I’m not a medical professional and don’t know if these drugs are appropriate for your mom’s situation. But this may be worth having a consultation with the hematologist oncologist to see if there is another treatment to be tried.

A bone marrow transplant is the only potential cure at this time. In the US there isn’t a defined cut-off age for transplant. I had mine at 65 and I know of other transplant patients well into their 70s. If your mom is in otherwise good health, at 67, statistically she isn’t too old for a transplant…at least age-wise.

Here are the links to information about PV with evidence of blast cells.

~From Medpagetoday.com
https://www.medpagetoday.com/resource-centers/polycythemia-vera/polycythemia-vera-blast-phase-new-findings/1940
~From PubMed:
https://pubmed.ncbi.nlm.nih.gov/29285580/
I think it’s worth talking with your mom’s doctor about what’s next. Has the doctor discussed any potential future treatments?

Our son was diagnosed with Polycythemia Vera Primary at 38 years old. He is 61. His spleen is enlarged so a transplant was not recommended. He has phlebotomies every 3 weeks. He has lost 30 lbs and is tired much of the time. His attitude is amazing, always a smile and we have tried to be encouraging.
There are several newer drugs that have promising results. He has chosen not to take them.
There are so many different ideas , suggestions, medication but you can’t stop the scoring of Bone Marrow. Each day is a gift
Embrace it. We have prayed for a cure and the scientists are working every day for a breakthrough. Continue loving your Mom and comforting her.
If she can walk it is good exercise and helps circulation. She must drink plenty of water , Warm showers only not hot.
I am a Mother with Polycythemia Vera secondary., and know how precious life is
Blessings to you and your Mother