Primary or secondary PV?

[Imkerin]

Hi everyone, as always really grateful for this Forum and the wealth of support and information it provides.

Brief background to my question; diagnosed PV February 2021 and tested positive for JAK2. Recently diagnosed with large mass in one lung that is obstructing airways reducing left lung function by 50-75%. This mass seems likely to have been slowly growing for at least two years and increasingly restricting oxygen supply. It is extraordinary how the body adapts and the right lung has (unbeknownst to me) been doing a great job to compensate and keep me upright.

Surgeons seem hopeful regards removing the mass and there is no spread outside the lung.

My question to the Forum is, is there a link between these two diagnoses? I understand secondary PV can be a consequence of chronic pulmonary disease. Is it possible to be JAK2 positive and have secondary PV?

As previously posted. The increase in breathlessness experienced over past six months was initially understood in the context of having PV and very low iron levels. It was only in the course of being treated for pneumonia (a consequence of obstructed airways) that the lung mass was discovered – now understood to be the primary cause of the breathlessness.

[hunter5582]

Sorry to hear about the mass in your lungs. Do they know yet what it is?

To answer a couple of your questions. The JAK2 mutation can cause erythrocytosis, which is what we see with PV. Obstruction in oxygen intake causes hypoxia, which triggers the body to make more red blood cells. When you already have PV, this can make the erythrocytosis worse.

There may be a direct or indirect link to the lung mass and the JAK2 mutation. The JAK2 mutation creates a pro-inflammatory environment in the body. We are prone to a variety of other neoplasms beyond the overproduction of blood cells. Not all doctors are familiar with this issue. While is important for you to have a MPN Specialist involved in your MPN care, it is critical to have one involved in care for issues like the lung mass. The MPN Specialist can give input to the pulmonologist to ensure your receive optimal care.

All the best moving forward. Do please let us know how you get on.

[Imkerin in reply to hunter5582]

Many thanks Hunter. The mass is 'highly suspicious' of being malignant. A second biopsy attempt due next week which will hopefully provide more information. It sounds grim but actually I'm relieved to have an answer to why I have been feeling so unwell and there is hope that with treatment may feel better. I am under an MPN specialist team and I have been surprised how they and the thoracic team are currently not that interested in communicating with each other. I will persist as I simply cannot believe that these two health events are unrelated.

[hunter5582 in reply to Imkerin]

Sorry to hear that the mass may be malignant. This is not s surprise. Your instinct that things are related is spot-on. The link between MPNs and secondary cancers is well established. It is related to deregulation of the JAK-STAT pathway. It does more than increase hematopoiesis. It also increases the risk of tumorigenesis and interferes with apoptosis.

Here are just a few relevant links to information.

ashpublications.org/blood/a...

ashpublications.org/ashclin...

ascopubs.org/doi/abs/10.120...

I have had to have multiple surgeries in the last several years, including heart and brain surgery. I insisted on collaboration. "Not interested" was not an option. We often have to be our own case managers in this situation, particularly if the MPN Team and the Thoracic team are not in the same hospital system. This is a case where assertive patients receive higher quality care. Passive patients do not. Collaboration is the key to successful treatment in this situation. Suggest you "assist" them in collaboration.

Do please stay in touch and let us know how things go. All th ebst to you on this next stage of your journey.

[Aime]

Sorry to hear of your illness. Hunter has given you very knowledgeable advice. Just to let you know I’m thinking about you and have my fingers crossed it is a good diagnosis. E hugs, Aime 😻

[Oscarsboy]

Very sorry to hear about this added burden you have to deal with. I wish you well with your upcoming surgery and follow on treatment. May you get all the care that you need between the teams dealing with it and blessings for a full and total recovery.

[Aldebaran25]

Best of luck , I hope you will be feeling a lot better after the removal of the lump and that your lung may recover quickly . Fingers crossed it will be an isolated matter.