Jac 2

[Chinaplatejules]

hi everyone, I have had mpn for about eight years now and have all the heat problems and very sensitive skin…I use bamboo sheets and nighties etc, cut tags off all clothing, I feel like a bit of a princess but there you go, anyway mine is Jac 2 does anyone else have this….truly I still have problems understanding all this mpn business so I would be happy to hear from Jac people

[hunter5582]

The JAK2 mutation is the most common of the three MPN driver mutations. About 95% of people with PV have it and 50% of people with ET. There are two known types of the JAK2 mutation, JAK2v617f (the most common) and JAK2 Exon 12.

The JAK2 mutation causes progenitor blood-making cells to self-phosphorylate. The on-switch is always on. This is why our bodies make too many of different types of blood cells. It is not well understood why some people make only too many platelets (ET) or too many Red Blood Cells (PV). Also note that some people with PV also make too many platelets and White Blood Cells too.

The JAK2 mutation does more than make the body make too many blood cells. It also causes the body to make too many inflammatory cytokines, this is thought to be the reason why we experience so many secondary and constitutional symptoms. Systemic inflammation causes a host of problems.

The JAK2 mutation upregulates the JAK-STAT pathway, which is one of the body's important regulatory kinase pathways. The JAK-STAT pathway is responsible for many things in the body. This is also why we are prone to other neoplasms and a variety of other issues.

Note that not all of the progenitor cells (Hematopoietic Stem Cells) are mutated. Some are wild-type (normal). This is referred to as Mutant Allele Burden or Variant Allele Frequency. Allele burden is associated with disease status, progression, and symptom burden. Note that this is a tendency - not a pure linear relationship. Also note that JAK2 mutated cells have a clonal advantage over wild-type cells. Allele burden tends to grow over time. People with ET tend to have the lowest allele burden, people with MF the highest, and people with PV somewhere in the middle. Emphasis on the word tend.

There is more to learn about the JAK2 mutation and how it works. The role of allele burden is a topic of active research. This is particularly important now that we have drugs like Besremi that appear to reduce allele burden, possibly inducing complete molecular remission (no detectable JAK2 mutation).

Here is an excellent presentation on the topic of the JAK2 and other driver mutations.

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

[saltmarsh in reply to hunter5582]

Hunter - many thanks for posting that video. I found it to be very informative and answered some questions I had. You're a valued contributor to this forum. Stay well. Saltmarsh

[Mostew]

If you arnt already doing so try antiinflammitory diet . May help with getting hot

[KLCTJC]

I have went to bamboo sheets too! But my best investment was a BedJet! I can’t live without my bed air conditioner! It was worth the money as it was a splurge. But if I we are out of town, I miss it!! I just can’t take the heat at all anymore! Grateful I can still take hot showers but other than that I need air!😂

[Chinaplatejules in reply to KLCTJC]

I even have to cut all the tags off my cloths because they irritate my skin

[Barbiebreath]

Me too China… I have to turn certain shirts and/or pj’s inside out… I sometimes just have to be very still until I fall asleep and I don’t want anyone touching me! It stirs up my itching. Such a crazy disease. Looks like I need to get some bamboo sheets!

[Chinaplatejules in reply to Barbiebreath]

hi Barbie they are brilliant but ____don’t buy the polyester mix just buy bamboo or bamboo and cotton, the other mixes are terrible