Prefibrotic Myelofibrosis: Diagnosis Favors Essential Thrombocythemia

My heart goes out to you Posted by mtnlife @mtnlife, 6 days ago. I don't know how to tell my autistic son (age 23) on 4th grade level that I have cancer and I turned age 51 (hospital for month in 2020 and they missed PV and Diagnosed with Myelobrosis Dec 30, 2022.) Doctors and more doctors - no one wanted me until I got cancer. Doctors didn't want me when I had rare life-threatening weird wacky symptoms and diseases. Now, everyone wants my blood - 3 doctor's apt. this week and can't keep awake but when I had then energy; doctors didn't like something they don't know, doctors like cold/ flu/ COVID 19. It's hard to be without family and symptoms. My neighbor Dave moved to be closer to family, he keeps being hospitalized with ulcers. At 71, moving is extremely hard and all lone. Wish I could help, prayers and best wishes (hope you find neighbors to help)

I was initially diagnosed with Essential Thrombosis ET be Suze of higher than normal platelets, rising from the 400s to 511, 588 then 612 in a period of a few months. I agreed on Nov 8 to take 500 mg hydroxyurea HU and in December had the bone marrow biopsy. BMB. My platelets have dropped to 399 but BMB revealed I have prefibrotic myelo fibrosis. Next step was ultrasound of spleen and not sure if it has enlarged or not. The interesting part is I have not experienced any definite side effects from HU and sailed painfree through the BMB. I am 83 and in good health up until now. At my age I expect to feel tired and am sometimes short of breath when climbing stairs or carrying heavy grocery bags. Watching tv and playing games with my grandsons ages 8 and 3 are my activities. I’m not sure what the future holds for me. Trying to avoid negative thoughts. Hoping the worst scenario doesn’t happen.

Thank you for the responses. I got through the BMB with minimal discomfort. Now awaiting the diagnosis. Trying to stay balanced and positive but not knowing is certainly hard.

Thoughts and Prayers! You got this!❤️🤗❤️

Okay, so I have a diagnosis, but it really is not that clear. The pathology report from the BMB "favors essential thrombocythemia". However, some mild to moderate fibrosis was detected. My hematologist says that this is generally good news, but that bloodwork will be monitored closely going forward. At this point I am on "watch and wait". The risk of thrombosis is less with the CALR mutation than with others and I am already on Eliquis.
I find this diagnosis a bit unclear and somewhat unsettling. I would welcome input from others with similar experiences. I do realize that the MPNs are extremely rare and so there just may not be others on this site who can related.
Beth

Hi Beth,
I moved your message about the update on your diagnosis to your original discussion. I did this so you can stay connect with @chrismary1224 @flynnconn and @raremiracle2. I'm sure they'll appreciate the updated on the pathology "favoring" the diagnosis of essential thrombocythemia.

You might also appreciate joining these related discussions:
- Essential Thrombocythemia: Looking for information and support https://connect.mayoclinic.org/discussion/essential-thrombocythemia-1/
- Essential thrombocythemia https://connect.mayoclinic.org/discussion/essential-thrombocythemia-26a26f/
- Myeloproliferative neoplasm Anyone else have a similar diagnoses? https://connect.mayoclinic.org/discussion/does-anyone-else-have-a-similar-diagnoses/

How are you doing?

@colleenyoung and @mtnlife and @chrismary1224 and @flynnconn Thank you Colleen young: any up date would be a blessing, nice to feel a connection with those who are going through the same thing. Thank you for all the information, experiences and roll-a-coaster ride! Truly is a blessing to have support and compassion, understanding and help to keep the faith! We are here together going through this journey together! Anyway: got my ultrasound (born with spleen defect (1/2 the size) today = normal size 14 cm and hepatosplenomagaley is 22.6 cm and normal is 7 cm -10 cm. Maybe it was good thing I had small spleen, so it can double in size (?think) Anyway: phlebotomy is Thursday. See if it helps my high blood cells counts. YES! HOW IS EVERYONE DOING?

I am enjoying warmer weather here in the middle Atlantic states and just took a two-mile walk. Lovely. The snowdrops are in full bloom and soon the daffodils will be showing their pretty blooms. Grateful to be able to enjoy this.

Hi Beth,
My experience is very similar to yours. BMB in September 2020 favors ET with Jak2 V617f mutation but Moffitt oncologist and local oncologist tell me that it could be ET or pre fibrotic MF as my BMB also shows level 1 and 2 reticulin fibrosis. I go for bloodwork once a month and all seems to be ok for now. From what I understand from my docs, MPNs are progressive and difficult to differentiate. I have read many articles from reputable websites that say this could all be one disease in different stages. The bottom line is that no one really knows for sure.
I know it's difficult, but the best things you can do for yourself are stay positive, exercise daily and take care of yourself. Dwelling on it will not help. The exercise will, both physically and mentally! As my Moffitt doc said, "It doesn't matter what we call it. It is what it is." At the time, I thought that was really harsh and lacked compassion. I now realize that he had a point. I can spend my time taking care of myself, or I can sit around and worry about it. Don't get me wrong, I do worry about it, but I don't dwell on it and let it consume me.
I hope you (and I, as this is my first post) can find the support we need to stay positive and learn how to live with this uncertain diagnosis.
Rayna

I was started on HU in November to lower platelets, but have experienced extreme fatigue, shortness of breath. Mouth sores and hair loss. I’m 83 and in otherwise decent health. I want to stop HU. Dosage has been 500mg daily. Platelets dropped from 612 in November to 270 now. Had bone marrow biopsy and the diagnosis went from ET to prefibriotic myelofibrosis Dr doesn’t want me to stop HU. But I agreed to do 3 times a week to see what happens. The side effects are bad for my everyday life.
PS Just noticed my previous comment has been moved here. I really don’t want to keep taking HU. Is this foolhardy? For some reason my dr wants me to continue. And before I insisted and made a fuss he didn’t even want to monitor monthly. He scheduled me for 3 months!! I feel very unsure about what is happening to me. He told me not to read anything on internet and just forget about everything and not worry??!!?? Result: I’m very unhappy about everything to do with my diagnosis