Cytoreductive therapy: Hello everyone,it's been... - MPN Voice

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Cytoreductive therapy

keviekevstar profile image
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Hello everyone,it's been ages since I've been here. Just wanted to ask a question. Spoke to my consultant and wants to discuss starting me on cytoreductive therapy as the venesections can't carry on indefinitely and are making me more iron deficient. Anyone had/ having this therapy. Are there side effects?

Many thanks

Kevin.

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hunter5582

Cytoreductive therapy is the use of medication to reduce hematopoiesis. Controlling erythrocytosis is the key element of treating PV, The target for males is HCT<45%. For females it is HVT<43%.

Many people are able to use venesection-only to control erythrocytosis, particularly those under age 60. The goal is to ultimately induce iron deficiency since this deprives the body of the iron needed to make RBCs. It is a trade off between the impact of chronic iron deficiency (without anemia) and the inherent risks of the medications (which have their own risks/adverse effects). I did venesection only for several years, but ultimately could not tolerate the iron deficiency.

There are two first-line cytoreductive treatments for PV, PEGylated Interferon and Hydroxycarbamide (hydroxyurea). Jakavi (ruxolitinib) is a second-line treatment option (better for certain symptoms like pruritis).

Hydroxycarbamide is the fastest acting of the three meds. It is also the cheapest, so preferred in some formularies. There are inherent risks in long-term use so it is generally not preferred for people younger then 60. It is a "highly toxic medication with a low therapeutic index." Still, it is effective and some people are able to tolerate and benefit from it. I was not. I experienced toxicity even at very low doses.

PEGylated Interferon takes longer to have impact for most people, but is the only medication that is potentially disease altering. There is evidence that it can help prevent progression of the MPN. For some, it reduces the mutant allele burden. Some people experience complete molecular remission. Most MPN Specialists PEG for younger patients. Many now prefer it for all patients. I am currently using Pegasys. At 45mcg/week it is controlling the erythrocytosis and thrombocytosis. I have experienced no adverse effects at all. Do note that PEG can have significant adverse effects. Some people cannot tolerate it.

Jakavi (RUX) works in a different way, inhibiting the JAK-STAT pathway that is in overdrive due to the JAK2 mutation. It reduces hematopoiesis and is very effective at controlling certain symptoms like pruritis. It is the most expensive of all of these medications and can be difficult to access in some healthcare systems. I have no experience with it, but other on the forum have spoken of its effectiveness for them. RUX does have its own risks/adverse effects.

The mechanisms of action for each of these meds is different.

HU - chemotherapy/cytotoxin

PEG - immunomodulator

RUX - JAK inhibitor

The best thing to do at this point is to educate yourself about your treatment options so that you can make an informed decision. It is also critical to consult with a MPN Specialist. Most hematologists do not have the KSAs to provide optimal MPN care. Here is a list. mpnforum.com/list-hem./

Here ae a few resources to get you started.

legeforeningen.no/contentas...

onclive.com/view/added-cyto...

mpninfo.org/conferences/202...

youtube.com/watch?v=_lLNp3M...

keviekevstar profile image
keviekevstar in reply to hunter5582

Thank you so much. This is extremely helpful.

Imkerin profile image
Imkerin in reply to hunter5582

What a great summary, thank you Hunter. Have already printed out a copy in preparation for discussion with Hematology team next year when I turn 65 and the conversation will start regards cytoreductive therapy.

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