Chance of UP developing into Systemic mastocytosis?

[Jazzsinger77]

Does anyone know what the chance of adult UP developing into the systemic type? I always presumed it was unlikely but read somewhere on this forum that most cases of adult UP develop into systemic? This is something that wasn’t really made clear at the hospital. Was more a case of keeping an eye and telling them if I was experiencing any changing symptoms such as headaches, stomach/digestive issues etc. I’m now thinking that I will develop these in time which is a bit upsetting!

[Jess-UKMasto Partner]

Hi Jazz. I am feeling disappointed and a little angry about your experience at the hospital because it sounds like they may have at very least not given you enough information. (The same happened to me when I was first diagnosed with UP, but that was in 1989 and one would hope there would have been progress by now). You're right about most adult UP being eventually systemic mastocytosis. While children often outgrow their mastocytosis (about 60% do) or go on to just have UP still as adults, adult onset UP is usually in the context of SM. A diagnostic workup for an adult with UP would look at tryptase levels, palpate the spleen and liver to see if they are enlarged, include a full blood count to see that the bone marrow is happily doing its job, check liver and kidney function, and also consider whether a bone marrow biopsy is warranted to confirm a diagnosis of SM. There are varying views about when a BMB is called for, but if the tryptase is over 20ng/ml, anything funny with the full blood count or enlarged spleen or liver or there are symptoms of systemic disease it is more often performed. A bone marrow biopsy would show whether there are extra, funny shaped mast cells in the bone marrow and would assess whether those mast cells are causing any change in how the bone marrow can do its job. The vast majority (at least 90% according to the studies I've see) of adults with SM have indolent (slow growing, lazy) SM and will live a normal life span with or without symptoms. Happy to have a longer discussion with you about how to ask for an appropriate work up from the hospital! And sending you a hug.

[Scotkiwi61 in reply to Jess-UKMasto]

When I was diagnosed with Cutaneous Mastocytosis, my dermatologist told me that she believes that most adult onset Mastocytosis will develop into Systemic Mastocytosis and her haematologist husband agrees with her.I have an annual review which is scheduled in the Summer when my dermatologist is on leave and so I always see another dermatologist. The one who reviewed my condition last year and the previous year told me that it is very unlikely that I will develop Systemic Mastocytosis!

So, even within a dermatology department in the same hospital you can be told conflicting information.

The recent dermatologist has a lovely manner but I suspect that most of his patients are children and so he is not so knowledgeable about adult onset Mastocytosis.

I have considered searching for one of the papers referenced in one of our conferences to send on to him.

[Jess-UKMasto Partner in reply to Scotkiwi61]

I agree with you that the second dermatologist likely sees children and doesn't have experience with adult onset mastocytosis. While there is still some disagreement in the medical community about whether ALL adults with adult onset mastocytosis have SM, even those who argue that some have skin-limited disease think it's a small percentage. Happy to think about an article for you to pass along if you'd like! You can drop me an email at [e-mail] if so.