Very long story short; listening to and acting upon amazing support and advice at the MPN Voice conference in Liverpool this September, I contacted Dr T S at The Christie hospital and had an initial appointment with him on 20th Nov. It was a very involved appointment where I finally felt listened to (it has become apparent I've been receiving a very mis-managed treatment plan all these years at my local NHS haematology clinic).
However, I've been left feeling confused .... I've been told, quite assertively, I do not have an MPN (i.e. because the BMB test I had a few years ago was JAK2 negative) and should not regard myself as having one.(an MPN).
'in order to have a diagnosis of myeloproliferative neoplasm one needs a mutation and currently we do not have one' (my follow up letter from clinic)
Which I sort of understand / have always understood I do not have Primary PV. So my question is..... should I no longer be classifying myself as having a "blood cancer"? My GP has just very sternly refused to use the term 'blood cancer' on my recent 'sick note/certificate'; "there's no mention on a diagnosed blood cancer on that letter" (GP comments).
I always thought secondary PV was also classed as a blood cancer. Am I / have I been getting it wrong all this time?
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MancMermaid
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I don’t think you can have secondary Polycythaemia VERA but you can have secondary Polycythaemia. My understanding is:
Primary Polycythaemia i.e. Polycythaemia Vera, is overproduction of blood cells (usually red blood cells but may also involve other blood cells) driven by a genetic mutation such as Jak2, CALR or Mpl and is an MPN i.e.a blood cancer.
Secondary Polycythaemia is not the same as Polycythaemia Vera and is instead an overproduction of red blood cells in response to something else happening in the body eg excess bleeding, COPD, sleep apnea, smoking, being at altitude etc. This can also be serious but isn’t a blood cancer and will often resolve when the underlying problem is addressed.
So I've been getting this wrong for all this time...... I shouldn't be saying I have a blood cancer? I'm so confused. I feel terrible / a fraud (probably shouldn't even be on this forum then?!)
No don’t feel like that - you need clarity and treatment. Your clinic letter says ‘we do not currently have’ - did they test for the other 2 known driver mutations (calr and mpl) or just Jak2? It may be that they will now want to run further genetic tests to see if you have one of those other driver mutations. (I believe there is a smaller subset of patients that are suspected of having an MPN even without one of these three known mutations but I’m hazy on that and not sure whether it’s because they think there could be driver mutations still to discover or whether because things like EPO levels indicate the cause isn’t Secondary to something else - others will be clearer than me re. this).
If it’s Secondary Polycythaemia it also needs careful treatment and to be managed or fixed to avoid risks from thick blood. It isn’t clear why this wouldn’t have been addressed during your earlier treatment if it is secondary.
I would contact the specialist’s secretary (or your GP) and ask directly for clarification and advice on what happens next (test for other mutations? uncovering and treating the cause if it’s secondary)?).
Wish you all the best getting clarity asap - it’ll give you peace of mind and get you the right care either way. (I saw your other post and you aren’t wasting anyone’s time - of course you need to know what you have and what the plan is. The Christie staff will understand this - these are natural questions. Good luck getting the answers quickly.
It would be correct that Secondary Polycythemia is not a blood cancer. It is a reaction to another medical condition, typically one that causes hypoxia such as chronic lung diseases, sleep apnea, congenital heart diseases. There are also EPO secreting tumors and other causes such as living in high altitudes. There are also some very rare conditions such as hereditary erythrocytosis. Diagnosis of Secondary Polycythemia requires diagnosis of the underlying condition causing the erythrocytosis. Treatment involves resolving the underlying condition. You should have that diagnosis in order to confirm a secondary polycythemia. Polycythemia.emedicine.medscape.com/arti...
Polycythemia Vera is caused 95%+ of the time by one of the JAK2 mutations. There is a small number of cases of PV that are triple-negative, no detected driver mutation but meeting all of the other diagnostic criteria. Treatment for PV requires control of the erythrocytosis through venesections and/or cytoreductive mnedications. emedicine.medscape.com/arti...
The statement that "'in order to have a diagnosis of myeloproliferative neoplasm one needs a mutation and currently we do not have one" in not technically accurate. While uncommon, it is possible to have a triple-negative PV (Meeting two major criteria and the one minor criteria). Note that if the BMB did not reveal the required bone marrow morphology and no JAK2 mutation or no low EPO, then the diagnosis would NOT be PV.
Here is a bit of information on this topic of triple-negative MPNs.
It sounds like you believe that you do have a Secondary Polycythemia, which is NOT a blood cancer; however, it sounds like you have some confusion about what this means. Suggest that you go back to your care team for a definitive answer to your questions. One of those questions, if you do not already know, is "What is the primary condition causing the erythrocytosis?"
I'm quite sure that is wrong. I don't have any mutations and yet I have both MDS and MPN, and have been told by my haematologist that it is a rare blood cancer. People don't understand those terms so I just say I have blood cancer, which is cancer with a small c. As I understand it, Polycythaemia Vera is a worse form of blood cancer than what I have. Just reading "Round the World" there may be more to it in your case. But I don't have Jak2 or any other mutation, or at least, none has been found. I would have thought "secondary" would be more serious, but what do I know?
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UPDATE: FIRST HAEMO APPOINTMENT. NO PV. 
ET jak2 to PV
PV - Tight Chest
ET? PV? Neither?
