Wendy, we all understand the confusion and we ourselves have these very same questions!   Did you know that there are cases of aggressive SM which the patient died from their disease without ever knowing they were even sick?!  I honestly can't figure that one out cause I'm forever taking a beating and although we're convinced that I have the neoplasm hidden in my body somewhere, we can't find the goober anywhere!!  My doctor's bald from pulling all her hair out over my case!!  She says she knows my case better than any of her other patients due to all my updates for all that I'm constantly reacting and yet, her other patients are all SM or UP patients and yet I give her the most trouble!!!  WHY?!!

According to the recent research coming out, Mast Cell disorders have now been found to be clonal - all forms of them, inclusing MCAS (MCAD - these terms are interactive only because there was no official designation between the S of syndrome and D of disorder.  MCAS is now the official standing)  Until 2007, there was only one recognized form of mast cell disorder, Systemic Mastocytosis, which included in that was UP Urticaria Pigmentosa and CSM Cutaneous Systemic Mastocytosis.   It was about this time, however that Dr. Akin rocked the masto researcher's world by deciding to study IA Idiopathic Anaphylaxis patients.  He was at the NIH at that time and he took a group of these patients and put their bone marrow through a very refined process and when he looked and tested for the clonal genetic defect HE FOUND IT!!  1/3 of the patients he studied had the defect and these patients didn't have ANYTHING else proving mast cell activation - neither raised histamine, tryptase or prostaglandins and they didn't have any pathological damage in their marrow either.  The ONLY thing wrong was that there was the very same genetic defect on their mast cells as the SM patients had.  

Well, since then, more of this same sophisticated testing has been done on the patients who did not fulfill the WHO criteria for SM and they are finding that there are different defects on the mast cells and they are beginning to feel that depending upon the genetic defect that this may influence how the disease shows itself and behaves.  

This is what gave to those authorities who believed that the MCAS patients were indeed mastocytosis patients, only with perhaps a different form, or perhaps were in a pre-SM state that these patients were still mast celld disorder patients since they not only behaved like SM patients but that they also responded to the same identical treatment.

You see, Wendy, what happend is back about 25 - 30 years ago two major doctors, Roberts and Oats of Vanderbilt University had come to the conclusions that there were two possible forms, that of a proliferative form and that of an activation form.   Researchers found the proof with the proliferative form but nobody could prove the activation form and they still can't.  They know something is wrong and so what happened is that the researchers made a mistake in concentrating all of their efforts and theories on only the one group that they could prove had something wrong and they excluded the patients who had no proof of this activation as to having something else wrong with them.   This is why when 2 years ago the WHO committee finally recognized MCAS a whole bunch of patients gave out a cry of victory for finally there was something for their doctors to go by, for without official recognition, our doctors didn't know what to do with us or how to treat us, even though it was that old saying " What looks like, smells like, acts like, but isn't like....?"   The only problem is that we are like, but there is just enough difference to make the researchers question WHY?!   This is the mistery they are trying to solve now for even though they still can't prove what is making the mast cells act badly, they have found enough patients who are not fulfilling the SM WHO criteria who have proof of the genetic defect to know that yes, these are indeed masto patients.  

For example,  I am suspected of the MMAS form.  This form is known to be clonal and they have found the genetic defect and have even seen that this patient has pathological damage which is in line with what SM will do.  This patient, however, has a low tryptase, but yet they will show elevated histamines or prostaglandins, thus proof of mast cell activation.  Why is the tryptase of this patient low, however?  Yet, why can't they find the MC neoplasm?  This patient also shows no urticaria or angioedema and yet goes through severe cardiovascular events and syncope as a distinguishing feature.

This patient ties together the two camps of clonal and non-clonal masto patients.  The WHO consensus on MCAS was that there are non-clonal MCAS patients and these patients don't seem to have any proof of the neoplasm.  However, some of the more recent research coming out shows that even though these patients don't seem to have the same kind of damage going on with them that the SM patients do, they do have many of the same issues - liver damage and bone marrow damage.  Yet, if this really is a non-clonal disorder, then why is some of the same damage going on with these patients, they really should only be going through the activation symptoms and not the pathological damage - logically reasoning it out.  Yet, this is what the researchers are questioning and why they are beginning to suspect that these patients are indeed clonal with their mast cells, that the genetic defect exists, however, due to the type of the defect on their mast cells, this may be what determines the degree of the form of the mast cell disorder, that the greater the defect, or the type of the defect, this will influence why one patient will be an ASM patient, another have a mastocytoma, another with leukemia, another with MCAS, another ISM, another MMAS and who knows what other forms may exist.   They just don't know yet, Wendy, and they just aren't certain yet.   They've not been able to prove yet the activation of the mast cells and those which they have been able to pinpoint show a genetic defect.  They are beginning to see that some forms may be just so incredibly slow growing that they never really are able to find the MC neoplasm and this would explain why these patients have so low of a tryptase, for what they've found is that the tryptase level reflects the MC burden and this would explain why some patients, without the sophisticated testing, will test negative for mastocytosis.  

Anyway, all of this is still so up in the air that unless you are reading the recent research coming out or speaking with the researchers, you won't find a firm stand on this and depending upon which camp of doctors you speak with, you'll find a different reply.  It's still a very wide open area of research and for now, it's still anybody's guess.

Now, why is it that you are seeing more MCAS pateints here?  Well, if you talk with the TMS site croud you will find more SM patients but let me say this - we who are MCAS are in a bad spot, Wendy.  The only doctors who are aware of MCAS are really into mast cell disorders.  Ordinary doctors barely will remember hearing about SM in med school and they've never heard of MCAS before and will openly refuse to recognize it due to how unknown it is.  The official WHO consensus document on MCAS came out only about 2 months ago even though the conference on this was in 2010!  It took them 18 months to finally agree and publish their consensus and that means that only those who are really into masto have a copy of this in their hands!   We patients must fend for ourselves until the medical community wakes up to this.  So, due to this, we have almost NO doctor support and very little community support and the only way we can find that is through the websites.  Depending upon the site, you will also find this divided camp of the SM versus MCAS group.   I was on one site where the patients had some really sick competition as to who was sicker, those with SM or those with MCAS and it was like those with SM won the prize because their bodies had more pathological damage due to the proliferation of the MCs into their tissues!  It was like it gave them more reason to complain whereas the MCAS patients were just playing at being sick!    I really couldn't understand this kind of attitude and I still find it totally revolting to think that there are those who seem to feel that it's some kind of status to say that they've got the one form versus the other!!  A sick kind of pride, if you ask me!!   This is why I can't accept putting any kind of initials behind my name as though it's medals to be proud of - I'm sure you've seen it people who will sign their names like Minnie Mouse ASM/ISM/POTS  and whatever else they put!!   I'm sorry, but I refuse to label myself and flash it about like Doctor will do with PhD or MD or DDS or whatever!   TOO WEIRD!

Now for the other part of your question on SM vs MCAS.  Do MCAS patients have more problems?   Well, I understand your question and I can't help but wonder that too, but I don't think so.  I've not got issues eating foods either, Wendy and I don't bother one bit with the low histamine diets! That would drive me nuts and I'm so grateful I don't have those issues.   Yet, up until last year I'd have told you that I don't have problems with smells and for the first 4 years after my masto came out of hiding I didn't have any problems with smells, but I do now!!  I was in the ER last week due to my husband having the car professionally cleaned - the silicone they used put me into a big attack!    Something changed, but I don't know what, nor how!!   Again, I think that depending upon the site, whether you have more MCAS patients or SM patients you will hear plenty of complaints.  The one site I was on that had more SM patients, I kept wondering what they were complaining about and they had all the same issues with food and smells that everybody hear goes through.  And yet, here is that same contradiction, there are plenty of case histories where you had UP patients who have had their spots for most of their lives and nobody knew what it was and yet they never bothered to consider it anything other than freckles until some doctor saw it and said, Hey, that's UP!  Those patients have either so few symptoms or are so mildly reactive that they'd never have gone looking for a disease!  And again, those patients who died from their disease never knowing they were deathly ill!!  There are several cases of where patients reacted on the operating table and surgery had to be stopped or they reacted to contrast and almost died only to have the reactions investigated and were found to have SM!   These cases are very common actually and it shows that there are plenty of patients walking around with masto whom nobody knows that they have it, that's how unsymptomatic they are.  

It really is a fascinating, contradictory disease, Wendy and this is also one of the curious things about it - that no two patients are alike!   It's a rule breaking disease and an extremely challenging one for doctors mainly because no two patients are alike!!  

Enjoy your uniqueness!!!!

Well, I hope this answered your question.  Sorry for the length!

Lisa

  As Lisa explained, while symptoms of allergies, MCAD, and SM might be similar, neither MCAD nor SM are allergic diseases, but they are diseases of part of the immune system.  Sometimes even doctors confuse them.

  Regarding the low histamine diet, only a few doctors recommend dietary restrictions, but all the ones I've seen say that there's no harm in trying.  A little background that some new people might not know....

  People with any form of mast cell disorder have too much histamine in their bodies on a regular basis.  Lots of things can trigger the mast cells to release histamine and other mediators.  Those triggers can be environmental (e.g., heat, mold, pollens, perfumes), but also foods.  

  Mast cell disorder patients can be exposed to some triggers, like stress or heat or a viral infection, for example, and we might only feel subtle symptoms or none at all until we get to the point where our trigger "bucket" is full.  At that point, just one more trigger can cause the bucket to "overflow", that is, cause our mast cells to release mediators and our MC symptoms to flare.  Allergic reactions are somewhat different from mast cell disorder degranulation, and yet some triggers and symptoms can be the same.  When mast cells degranulate, there are many different substances that can be released into the body, including granules containing histamine, heparin, prostaglandins, serotonin, and many more.  Each of these causes different types of symptoms.  

  The goal of eliminating triggers is to stop degranulation and thereby minimize symptoms.  Most people with SM and/or MCAD find they have food triggers, some of which they were not previously aware.  An elimination diet is one of the best ways to figure out what unique food triggers cause problema.  Elimination diets are also used to discover hidden food allergies in allergic people.

  Some foods cause direct histamine release in many people.  Other foods contain (or might contain) high levels of histamine, either naturally or during the process of decay.  For example, strawberries can cause direct histamine release in both people who have a true strawberry allergy and also in mast cell disorder patients for whom strawberries are a trigger.  

  On the other hand, e.g., tomatoes, aged cheese, and eggplant are naturally high in histamine, especially when ripe.  As soon as some fish die, the bacteria in their bodies begin to break down tissue and in the process can produce massive amounts of histamine.  In tuna this can happen within 1/2 hour.  This happens in meat, too, if it's been cooked and sits for more than about 4 hours, even under refrigeration.  Quick cleaning and freezing of fish keeps histamine from forming in great quantity.  Freezing meats shortly after cooking keeps histamine formation to a minimum.  Ingesting histamine can cause anaphylactic or anaphylactoid reactions.

  An elimination diet limits food intake to a few foods that are generally safe for allergic or mast cell patients.  A low histamine diet also eliminates foods that can cause direct histamine release or which contain high levels of histamine.  A by-product of these diets is that chemical additives, preservatives, and artificial ingredients (including food colors) are removed from the diet, as those things might be triggers, too.

  Using myself as an example, I decided to try the low histamine diet as a starting place to try to pinpoint what was causing my symptoms besides environmental and emotional factors.  On any elimination diet, it should be followed religiously for 10 days to a few weeks before trying to add back in other foods.  What I found was that if I stopped eating leftovers that hadn't been frozen and stayed away from high histamine foods, that my symptoms were much better.  I found food groups that can cause me problems, and then I found foods that I could add back into my daily or weekly diet and didn't increase my symptoms.  At this point, I've found that I can eat most freshly cooked or quickly frozen foods.  Certain things will bother me, especially foods high in histamine.  Some foods I rotate in and out of my diet, but if I have them several days in a row, they'll trigger me.  I have enough variety of foods I can safely eat so I only sometimes feel angry and resentful about the restrictions.  

  Some people can figure out part of what bothers them by eliminating food groups often known to cause problems.  For example, they can cut out all dairy and see if symptoms improve or if that doesn't help, they can cut out everything with salicylate or tyramine or high acid.

  The low histamine diet published by ICUS for people with chronic hives seems to be a good starting point for some mast cell disorder patients:

[url]www.urticaria.thunderworksinc.com/pages/lowhistamine.htm/url]

  I often suggest it for people who are on what should be sufficient amounts of antihistamines, but who still have uncomfortable symptoms, and for people who haven't yet been able to reach a therapeutic dose of antihistamines to stop their symptoms.  At this point, cooking only fresh foods from scratch using mostly allowed ingredients has become routine.  I miss some foods I can't eat, but it's worth it because I feel so good on a daily basis when I avoid food triggers, and suffer the repercussions when I don't.  BTW, I don't have any true food allergies at all, just food triggers.

  Still, it's a real challenge to limit foods when we feel restricted in so many other ways, and some people can't or don't want to try it.  Understandable.  Everybody has to cope with this disease in her/his own way.

I hate it too, Wendy.  If a restricted diet didn't help me feel better, I'd be off it in a heartbeat!  I don't have a great deal of willpower, but I've found a lot of substitutions to use in recipes, and I don't react to all the foods that are on the "avoid" list.  I do miss some things a lot, though, especially when my family can eat anything they want to!

Hi Peter,

Are you able to read this entire article, cause I can´t.  Is this the same diagnostic proposal article that Dr. Akin put out in 2010 by chance?  It´s hard to say if it is or not or if this is a web article that Dr. Akin did especially for this website.  

Also, I´m curious why you say that SM can be prevented.  I´ve not read that anywhere and since they are still not sure how it´s caused, how can it be prevented?  Can you explain please?

Thanks!

Lisa

hi maiysa i was diagnosed with clonal mcas- sm 4 miner
bot only 3 mast cell in the first bmb 12 mounths later small clusters of 4+2 so it was servire CMCA- MINAMAL SM so the doc sead

lisa i did have the full article on the web i see now that have stoped it
dont no why MCAS IS IDIOPATHIC CMCA AS PRIMARY WITH 2 MINER Critirea for sm

ha joan lisa was looking for the mast cell acativation one
it is now only avalabull for money it was very good but i would not pay for it