Hydroxyurea Dosage

[Kame279]

After many many years of no health concerns, my wife at 68yrs old last December, 2021 had a routine cbc and the platelets were at 2200 on the usual 150-350 scale and confirmed with a blood smear. No other blood parameters were interesting and all the typical genetic tests including the over 50 ngs genetics were all not detected. More blood tests were performed including many imaging scans all over the body to locate a possible condition. Nothing was found. A bone marrow biopsy found some indications of ET. She has been on HU and mild aspirin. The HU dosage is now at 500mg per day (one pill) and that has been yielding a count of 400 platelets per microliter of blood. The necessary dosage has been going down the last few months. We understand the treatment only reduces production. I am wondering about others experiences. As this is half the recommended dose by weight and the platelet count was so high, is their any significance to this? --thank you

[hunter5582]

Hello and welcome. It sounds like your wife has an unusual variant of a rare disease - Triple-Negative Essential Thrombocythemia. That makes it all the more important to consult with a MPN Specialist. Hopefully she is already seeing one.

You are correct to think that she has had an unusually strong response to HU. However, we all respond differently to HU and the other treatment options. HU did not work well for me and i could not tolerate it. I responded much better to the interferons (Pegasys/Besremi). My experience would have little to do with how she would respond.

Suggest you ask for an interpretation of her response to treatment and current status from a MPN Specialist, This doc can better put into perspective your wife's condition and response to treatment.

Meanwhile, here are a few resources you may find of interest.

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

researchgate.net/publicatio...

youtube.com/watch?v=Oc8ujrS...(Not working as expected?)

[Kame279 in reply to hunter5582]

Thank you for the quick reply and information. I have read hundreds of papers. With a physical science background it has taken some time to get a footing in life sciences. I had not seen those two papers. In perusing them, allowing for variation in terminology, I believe those refinements have been tested and we have not begun the process of questioning some results. Immediately, I was more concerned that with the high count making it easy to overlook conditions outside the bone marrow or concurrent ones that maybe overlooked. I think that has been done well enough for now. There is a cancer doc, but I agree we are getting close to needing someone that just does this condition. The thought was to wait until it was about one year and spend the time trying to identify that specialist. About the hu dose. Initially it was high to look for a reaction. About six months ago it was at 1000mg per day to get a reduction near normal. In the last two months it has descended to 500mg to achieve the same level. That just seemed odd but maybe not significant to et. Being math people, we have this all graphed and modeled. lol thanks again.

[hunter5582 in reply to Kame279]

You are absolutely correct that your wife needs to consult with a MPN Specialist. Most hematologists/oncologists rarely treat MPNs. They simply do not have the KSAs to provide optimal care. I actually use two hematologists. One is a MPN Expert doc at Johns Hopkins, about 2 hours away. This doc provides MPN expert consultation and helps guide my care plan. The other doc is a wonderful local hematologist who provides the ongoing care. This arrangement works great. Just in case you have not seen it, here are two lists of MPN expert docs. Note: there is often a wait to get into to see these docs.

mpnforum.com/list-hem./

pvreporter.com/mpn-speciali...

I agree about being a numbers person when it comes to tracking MPN status. It is the trends over time that matter, not any single lab read. I am fortunate that my patient portals permit me to print a historic view of any lab we track. It is very easy to see the trends in chart form.

I would also agree about monitoring for co-occurring medical conditions. There are definitely conditions that cause reactive thrombocytosis. MPNs cause more issues than just too many blood cells. People with MPNs are more prone to other neoplasms. The deregulation of the JAK-STAT pathway also causes the body to make too many inflammatory cytokines. At the core, MPNs are inflammatory disorders.

It is really good news that you have a background in physical science. Understanding MONs requires a solid background in physical science too. The training is research methodology also helps to interpret the studies you read.

All the best to you both.