Polycythemia Vera

Posted by atir @atir, Nov 30, 2018

Have been diagnosed with polycythemia Vera recently, Any feed back

@gjb1

Hi,
Just diagnosed with Polycythemia Vera on Friday. Negative JAK2 test, but other genetic changes noted on bone marrow biopsy. Had my first phlebotomy done Friday as well. Hemoglobin is 18.8, Hematocrit 58.9. Have some of the symptoms, fatigue, bruising, shortness of breath, etc. My first EPO was normal, but late Friday got a repeat back showing high 61. Now I'm not to sure what's going on! Hopefully will speak with the Hematologist on Monday. Haven't gotten the repeat JAK2 back yet either. Just wanted to check in!

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@gjb1 – My EPO is high as well. In my case the high EPO makes sense, given my hematocrit is anemic. I'm still confused by my PV diagnosis, given now the only criteria I meet is that I have the JAK2 mutation. Feels a bit to me like an "eeny-meeny-miney-mo" diagnosis. It could be possible that I'm in early stage PV, but, currently there is no bone marrow biopsy to confirm that, and two of the bloodwork parameters (hematocrit/hemoglobin and EPO levels) are contradictory to the diagnostic criteria.

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@apr931

As a follow up to my question, I searched for diagnostic criteria for PV (since they kept emphasizing that DIAGNOSTIC criteria is a hematocrit over 32." They kept using "32" as some magical number. I know that internet resources aren't always accurate, but EVERY paper I have found in the peer-reviewed literature states that one of the major diagnostic criteria for PV is: "Hemoglobin >16.5 g/dL in men and >16 g/dL in women, or hematocrit >49% in men and >48% in women, or red cell mass >25% above mean normal predicted value" (reminder, my hemoglobin today was 10.7, and hematocrit 33, my red cell mass has never been studied." So, where did this magical number of 32 come from? And, does it make sense considering that value would make me anemic (not polycythemic)? Not sure if this is the case, but OLD criteria (from 1970 group), included as one of the major diagnostic criteria as "Category A criteria are as follows: Total red blood cell mass ≥36 mL/kg in males or ≥32 mL/kg in females" I'm wondering if I was literally just diagnosed with PV based on the WRONG measurement (as I have never had red cell mass measured), from an old set of criteria. This would explain the PV diagnosis, which is seemingly contradictory with my (albeit slightly) anemic hemoglobin/hematocrit. Any thoughts on this explanation, or, just in general about if/ow a definitive diagnosis of PV can be made based on just the JAK2 mutation and LOW RBCs?

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@apr931 Hi there! I wanted to chime in on your question regarding the number 32. I have no idea where that came from. As of your post that I am responding to, where your hemoglobin was 10.7 and hematocrit 33, both of those results are below normal. For a man and for a woman. Polycythemia vera diagnostic criteria includes one of which that is a highly elevated hemoglobin and hematocrit. Which you do not have. However, another diagnostic criteria for PV is the JAK2 mutation. I'm guessing your PV diagnosis was based solely on the JAK2 mutation, which would be inaccurate according to your hemoglobin & hematocrit levels. Throughout my extensive research into PV and myeloproliferative disorders in general, your particular results seem to point more towards essential thrombocythemia (ET), or primary myelofibrosis (PMF). Your low hemoglobin and hematocrit point away from PV. The diagnostic criteria for PV was recently-ish revised in 2016, and still your low hemoglobin/hematocrit levels point away from PV. Bless you & fight the good fight! Just trying to provide some (hopefully) helpful information. 🙂

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@apr931

@gjb1 – My EPO is high as well. In my case the high EPO makes sense, given my hematocrit is anemic. I'm still confused by my PV diagnosis, given now the only criteria I meet is that I have the JAK2 mutation. Feels a bit to me like an "eeny-meeny-miney-mo" diagnosis. It could be possible that I'm in early stage PV, but, currently there is no bone marrow biopsy to confirm that, and two of the bloodwork parameters (hematocrit/hemoglobin and EPO levels) are contradictory to the diagnostic criteria.

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A high EPO is indicative of secondary polycythemia. Those with polycythemia vera (primary polycythemia) have low EPO levels. Cheers! 🙂

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General 'normal' range for EPO is 4-19 mU/mL

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Also, I am in no way a medical professional. However since my journey began of being evaluated for PV in 2015, I have become a 'professional' researcher in the disease. I have spent countless hours researching since 2015. I am only trying to share what I have learned and I truly wish you all the very best.

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I’m a pediatrician working for more than 27 years.I have been diagnosed as a polycythemia patient 10 years ago with ability to mention either a primary or secondary
I did everything for diagnosis
Erythropoietin
Sonar kidneys
Bone marrow biopsy
MRI brain
Jack test
Everything and nothing is there
What I used to do is venisection every 3 month when hematocrit ratio exceeds 52 and when HB reaches 17gm
The best thing I noticed that vitamin C delays the intervals between blood donation

Now I’m taking Vitamin C 1 gm daily regulary for 6th months
Good news that HCT back to 48 without venisection for 2 times 3 months apart

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Do any of you who have been diagnosed with PV experience flushing of your skin? Specifically face, neck and chest areas. Sometimes spreading down the arms? Like this…

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@simple27

A high EPO is indicative of secondary polycythemia. Those with polycythemia vera (primary polycythemia) have low EPO levels. Cheers! 🙂

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According to the Hematologist, even a high EPO of 61 is still lower than what she would expect with secondary polycythemia from a tumor etc. Continuing to treat me as if PV with phlebotomy.

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@simple27

A high EPO is indicative of secondary polycythemia. Those with polycythemia vera (primary polycythemia) have low EPO levels. Cheers! 🙂

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@simple27 — Agree that my anemic hematocrit points AWAY from PV. I wish I could convince my oncologist of how ridiculous this diagnosis is. Almost like saying "you are obese because you weigh 80 pounds." I suppose it is POSSIBLE to have masked polycythmia vera, but that's usually only true if there is substantial, consistent bleeding. But, in any case you cannot DIAGNOSE PV BASED on a low hematocrit– it makes no sense.

My EPO levels also point away from PV, as they are high. So, yes, my brilliant oncologist diagnosed me with PV based solely on the JAK mutation, while I am the OPPOSITE of two of the other criteria, and have never had a bone marrow biopsy.

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@apr931

@simple27 — Agree that my anemic hematocrit points AWAY from PV. I wish I could convince my oncologist of how ridiculous this diagnosis is. Almost like saying "you are obese because you weigh 80 pounds." I suppose it is POSSIBLE to have masked polycythmia vera, but that's usually only true if there is substantial, consistent bleeding. But, in any case you cannot DIAGNOSE PV BASED on a low hematocrit– it makes no sense.

My EPO levels also point away from PV, as they are high. So, yes, my brilliant oncologist diagnosed me with PV based solely on the JAK mutation, while I am the OPPOSITE of two of the other criteria, and have never had a bone marrow biopsy.

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I just hope this isn't preventing you from getting the correct treatment/relief. I would imagine it does and understand your frustration. I have the exact opposite situation. High hemoglobin and hematocrit, low EPO but no JAK2 mutation. According to a DNA report that I just ran the other night, I do have variants in my JAK2 genes so I emailed my hematologist to ask if that would be helpful in a diagnosis. I'm pretty sure he doesn't like me much because I am VERY proactive in my healthcare. Have to be. I'm just a number to these doctors.

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@simple27

I just hope this isn't preventing you from getting the correct treatment/relief. I would imagine it does and understand your frustration. I have the exact opposite situation. High hemoglobin and hematocrit, low EPO but no JAK2 mutation. According to a DNA report that I just ran the other night, I do have variants in my JAK2 genes so I emailed my hematologist to ask if that would be helpful in a diagnosis. I'm pretty sure he doesn't like me much because I am VERY proactive in my healthcare. Have to be. I'm just a number to these doctors.

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@simple27 I took my promethease jak2 variant information with me to my Dr appointment. I’m showing three areas of increased risk. I’m now waiting for Jak2 lab results. Blood was drawn last Thursday (feb14) at Mayo, any idea on the turn around time for results there?

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@thlas1971

@simple27 I took my promethease jak2 variant information with me to my Dr appointment. I’m showing three areas of increased risk. I’m now waiting for Jak2 lab results. Blood was drawn last Thursday (feb14) at Mayo, any idea on the turn around time for results there?

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For everyone’s info, I messaged through the patient portal and was told up to 10 days for Jak2 results. But the nurse suggested I check in at 7 days in case it is available. Wonder why it takes so long.

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