Update on my polycythemia vera diagnosis

[Blonde25]

I have received my hospital consultant letter from the GP and I'm still trying to understand the blood counts etc .

Jack 2 positive polycythemia vera with paraesthesia .

Jak allele burden 20.83% EPO < 1

Spleen 11.2 cm

Bone marrow consistent with PV with MF 1/3

WBC < 12

Platelets <450, haemltocrit 0.50

Could anyone explain what MF 1/3 means ?

And if the platelet's are normal?

Should I ask for another venesection if my Haemotocrit is . 050 I thought it should be 45 for women ?

I am not being seen again until the 24 march and that's a phone call consultation after they receive my bloods that are booked in for the 20 march . I'm due to take my second peg injection tomorrow.

I'm still waiting for a brain and heart MRI scan has my echocardiogram detected a heart problem and the consultant think I may of had a mini stroke about 9 months ago 😩

[ainslie]

you should definitely get a venisection asap, I am very surprised your consultant or GP hasn’t insisted on that. Thrombotic risk rises exponentially as Hct rises. Men should be under 45 and women many believe should be under 42 (confirmed again by Dr Silver at recent conference in Phoenix/ MPN foundation) and Dr Spivak at Oct conference in NY.

Dont want to worry you but better safe than sorry, main cause of death in PV is not progression but thrombosis, so reducing Hct asap is a priority. Peg can take a while to control Hct so venisect in the meantime.

Allele burden looks quite good, platelets are fine as are whites. I have never seen platelets or whites expressed as less than, how about the actual number.

Spleen looks okay.

I don’t know what they mean by MF 1/3, maybe others here know but really this should be discussed with your Haem or at worst Haem nurse.

Good luck😀

[Blonde25 in reply to ainslie]

Thank you for your response, it's hard getting to know all of this when first diagnosed

[Manouche]

MF 1/3 means you have a stage 1 myelofibrosis which is quite reassuring as we all have a certain level of myofibrosis associated with our condition. I wouldn’t worry too much about your HCT at 50. It needs to go down to 43, but it’s not an absolute emergency unless it goes up next month. Mine reached 67 at diagnosis and was down to 43 before reaching 52 after a few months of low Pegasys dosage. It can take months to stabilize and the less venesection you have the easier it’ll be to get the Goldilocks number with Pegasys.

[ainslie in reply to Manouche]

sorry Manouche there is no point in sugar coating this but you are giving dangeroes advice, Hct should always be under 45 and ideally under 42 for women at al times if PV, any haem that knows anything will confirm, it shouldnt be at 50 ever.

[Manouche in reply to ainslie]

Hi ainsly, I’m not sure that making someone scared is appropriate in this case. Everyone would agree to say that a low and stable HCT is necessary for any PV patients in the long run. A transient high HCT during the dose escalation with interferon is generally considered non-problematic. Venesections tend to slow down and even counteract the effect of interferon on the hematopoietic system. Personally, I would trust her haematologist and wait until the next appointment in March. A good haematologist is treating patients, not numbers.

[ainslie in reply to Manouche]

sorry to disagree but its not my intention to scare anyone but its better to be safe than sorry, the facts are there Hct should be under 45 or really 42 for females, 50 is way too high, most deaths with PV are from thrombotic issues and its a fact thrombotic risk increases exponentially as HCT rises. The above fact were presented by Dr Spivak at the doc to doc conference in Oct in NY and again by Dr Silver at last weeks conference in Phoenix. These docs are the top end , I tend to believe them, at no point in their presentations did they say its okay to keep your Hct at 50 for some of the time. Hence when people are diagnosed they are usually venisected up to twice a week until Hct is under 45 or 42. I hear what you say and of course nobody wishes anyone to be unnecesarily worried but PV is a serious condition with risks and the guidelines are there for a reason ie to keep people as safe as possible.

[ainslie in reply to ainslie]

I attach the curve exponential increase in thrombotic risk as Hct rises as proved by Dr Spivak at his presentation this Oct 2022 doc to doc conference in NY. The full presentation can be viewed on Vumedi for those who have access. Apologies for poor image, my computer skills are not brilliant

Effect of Hct on thrombotic risk exponential curve

[ainslie in reply to ainslie]

Separate image showing similar

Hct and thrombotic risk

[Manouche in reply to ainslie]

You are absolutely right ainslie, but you get confused between statistics and individuals. Again, haematologists treat patients, not statistics. That’s why people from places like La Riconatado don’t get any phlebotomy despite a HCT above 60 (up to 80). Everybody is different and each treatment should be individualized.

ncbi.nlm.nih.gov/pmc/articl...

[ainslie in reply to Manouche]

I dont think I am confused but it looks like you have confused comparing non PV patients with PV patients , the paper you posted isnt really relevant as they dont have PV , which is quite important , if non MPN Hct over 50 isnt a issue, on my CBC results they quote up to 52 as being normal but not for PV patients

[Manouche in reply to ainslie]

Ainslie, normality is based on statistics. We are not statistics. Allow me to stop this discussion which is becoming quite circular…

[Snook37 in reply to Manouche]

I have found that if you follow RBC count, iron deficiency, and MCHC/MCH, and MCV it can be quite complicated. As iron deficiency reduces the MCH/MCHC goes up along with MCV, and the RBC may stay stable or reduce but HCT still goes up. These days the HCT is just a calculation, MCV x RBC count. I keep a close watch on RBC count, MCV along with HCT. The HCT is just a mathematical figure and the MCV and RBC counts vary in lab findings dependent on the day or minute potentially, and the lab, thus affecting hematocrit. To Manouche’s point, RBCs may be controlled, but there is a recovery in iron deficiency and thus MCV, which all by itself affects the HCT. A hematologist is best suited to synthesize all these moving parts.

[Elizka in reply to ainslie]

Regardless of the medication plan, most HEM's will tell you that the biggest mortality risk for PV patients is stroke or blood clots and a high HCT can cause both of those.

[hunter5582]

You really should get the exact lab values to interpret this properly. In addition, review your findings with a MPN Specialist. Managing a MPN is beyond the scope of practice of a GP. Most hematologists have little experience with MPNs, but could at least give a better interpretation of the results. Here is a list of MPN Specialists. mpnforum.com/list-hem./

Your profile sounds like a pretty typical PV case. Your erythropoietin (EPO) is low, while your hematocrit (HCT) is high. Your JAK2 allele burden is relatively low and pretty typical for PV. Results are Grade 1 fibrosis, which is not uncommon with PV. Platelets are in the normal range, no thrombocytosis. Harder to interpret the White Blood Cell count, but it sounds like there is no leukocytosis evident. It appears that spleen size is normal, no splenomegaly.

Definitely insist on copies of the actual labs and reports. This will allow for more accurate interpretation.

Regarding a venesection, your care team should already have ordered one at HCT > 42/43% female. It would be HCT > 45% for a male. This is especially important with a detected heart issue and possible stroke history. You absolutely insist on a venesection immediately.

Pegasys is a very effective treatment for many people with PV; however, it takes time to bring your blood counts under control. It is very common to need venesections as you initiate this cytoreductive therapy.

Suggest you do whatever is needed take to consult with a MPN Specialist as soon as possible. You deserve a comprehensive answer to the meaning of your labs. You also need guidance regarding an appropriate care plan for the PV as it presents in your case.

Please do let us know how you get on.

[Blonde25 in reply to hunter5582]

Thank you , the letter was from the hospital haematologist to my GP then they send me a copy . I will ask about venesection because I don't go back until 24 march . Thanks for the list of MPN specialists hunter my consultant is on the list of MPN specialist Dr Garg

[hunter5582 in reply to Blonde25]

I would definitely reach out to Dr. Garg's office to get the order for the venesection sooner rather than later. You may also request hard copies of your labs from that office. You should also be able to log into the patient portal and see them yourself.

[Ebot]

Hi there. My own experience of being diagnosed with PV when my HCT hit around 50 (having lived with unclassified MPN treated as ET for a number of years) was an immediate venesection followed by a repeat a fortnight later and then monthly until my numbers stabilised in the early 40s.

Bringing the HCT down is usually a priority because of the risks associated with numbers over 42 / 45 (and while this might not be such an issue in non MPN patients it is in those with MPNs).

So, I definitely think it is worth asking about this and finding out if venesections are part of your immediate treatment plan. It’s usually the first line. Drug interventions are not always necessary especially for younger patients with no other health concerns. However practices can vary according to haematology teams. My experience was with Prof Harrison at Guys.

Wishing you all the best.

[Looolooo]

When first diagnosed at 50 hct, I had weekly venesections to bring it down ASAP. Exhausting, but very necessary.

[Meatloaf9]

My MPN specialist has told me on several occasions that the only thing that is known for sure is that PV patients should keep their HCT below the 45/42 targets. Best to you.