Thursday, July 23, 2009

Do you have a question? Feel free to ask here!

I figured I would try something different, if you have a question you need answered just post it here and I'll do my best to answer it. Also if another reader knows the answer please feel free to jump in and help.


Anonymous said...

Hi! I am a 37 year old with indolent systemic mastocytosis. I'm really worried about mine turning into leukemia and my specialist (Cem Akin from the University of Michigan) is leaving my home state to work at Harvard University. I was curious how long have you lived with this? What is/was your tryptase level and do you know what it means if it keeps going up? I just can't get answers as I lack a good doctor for my disease here in Michigan. I thought by finding someone with experience to chat with would help me stop worrying. I love your blog and hope to chat soon. MastoinMichigan

Kevin said...

I suffer from Indolent Systemic Mastocytosis as well. I also have a concern regarding leukemia. I've lived with it since 1997.

As far as your typtase level: "Tryptase levels are continuously elevated due to the large number of mast cells."

I found this pdf article you may find interesting located here: let me know if it helps answer some of your questions.

I also found this that has a great deal of information regarding "Diagnostic Value of Tryptase in Anaphylaxis and Mastocytosis" located here:

I would love to chat with you anytime, if you ever wish to talk via phone just let me know and we can do that as well. My email is:

Hang in there, I know our illness can have it's rough moments but we still have a great deal to offer.

Anonymous said...


Del's Tales said...

Does Dr. Srdan Verstovsek only see systemic masto patients? I live close to MD Anderson and my 3 year old daughter has UP/CM. She has a new growth, possible tumor, on her back. Right now her Allergists do not know for certain what it is. Her move involved site is:
Thank in advance for any comments/information!

Kevin said...

Del to be honest with you I'm not sure. Only suggestion I can give you is to call him. If you give me your email address I'll email you his contact information.

I wish I could be of more help, I'm very sorry your little girl is having so much trouble. I have three girls so I feel your pain.

Mastomommy said...

My e-mail is:

Thank you very much! I appreciate it. We have 3 girls and 3 boys. She is our baby. She is such a good little girl! You would never know that she had anything like Mastocytosis going on with her unless you saw her have a reaction. She had a flare up start up yesterday but we were able to get her cooled down fast enough before it got to the full blown state! This heat has been just awful!

Carrie said...

I have struggled with gastrointestinal problems for years: diarrhea, nausea, vomiting, weight loss, painful radiating abdominal cramps. I saw a pediatric gastroenterologist when I was an adolescent (I am now 22) and was checked for Crohn's disease, ulcers, bacterial infections, etc. I am hypersensitive to foods (dairy, greasy or oily foods, high fat content foods) and sometimes get sick after heat and high activity exposure. Usually when I eat these foods, I get stomach pain that develops into diarrhea or nausea very quickly, sometimes before I am even done with the meal. I also was diagnosed with acid reflux disease and have unexplained bone pain and dizzy spells sporadically. When I was younger I used to get LARGE, red, itchy hives across my stomach area for unexplained reasons, but have not seen any in years. I was wondering if anyone has been diagnosed with systemic mastocytosis (particularly that affecting the GI) and would like to know how and if I should get tested for this disease?

Susan L said...


What type of information should I put on a medical ID alert bracelet? So far I have non-systemic masto but I am unsure what I should say about anesthesia etc. I have already had a severe reaction to an insect sting and want to order a bracelet and print out a card to carry. Thoughts?

Kevin said...

Hi Susan,

I put on my Medic Alert bracelet the following:

Systemic Mastocytosis
Use Anesthetic Precaution
Severely Allergic Preservatives

That is all that would fit, the cool thing about Medic Alert is they gave me a card for my wallet as well. Everyone has different needs just list the most life threatening things first so they appear on your bracelet, the rest of the information they’ll keep on file for when they are called by a caregiver if the need arises.

I would probably do something along the following:

Use Anesthetic Precaution
Severely Allergic to Insect Sting

Medic Alert will help you pick out the most important things and how you should list them on your bracelet. The card is very important make sure and list your caregivers, phone numbers, any other important contact information such as a spouse, mother. Of course you’ll mention all your medications, your major concerns and so on.

I’m sorry if I did not answer your questions completely, everyone is so different. I would visit with the people at Medic Alert first they have a great deal of experience in helping people list the most vital information.

Hope this helped,


Susan L said...

Thanks Kevin - I have no idea how you do cope with it all - I'm overwhelmed just thinking about non-systemic masto. I'm going for my yearly blood draw last week - hopefully things haven't changed but I feel like they have. I'll contact Medic Alert and get that bracelet ordered today. Thanks again. Susan in Texas

Kevin said...

Hi Susan,

What part of Texas? I'm in Victoria. You never get use to living with SM you just have to deal with it and do the best you can.

I hope your lab work comes back positive.

Susan L said...

Kevin -

I'm in Austin. I'll let you know how things go next week after the lab stuff is back. Thanks again- You are the first person I have talked to who has any form of mastocytosis. Everything I know about it comes from the web or my dermatologist, who is the one who found it.

Kevin said...

I've yet to meet anyone like myself, I've never been able to make it to a convention. If I can be of any assistance please don't hesitate to ask.

Take Care,


ginger said...

do you think i have masto? i have an appointment on the 16th with gastro, need to schedule with cardo next fri and seeing an allergist middle of next month...feel like i am falling appart.

History part 1
1997 I.C. symptoms, o bacteria in urine. Treated with sulfa drugs for the next several years. Admitted to the er, fever, bloody stools, vomiting. Told possible crones disease. On a soft food diet for a month and symptoms improved. Vomited if drank any alcohol.

1999-After giving birth hive break out off and on for 1 week.

2001- frequent nausea and vomiting.

2006- Car accident. I.C. diagnosis. New symptoms, sensitive to cold, dark patches on forehead which increased with sun exposure, fatigue, cholesterol (268), sinus infections, mouth and nose sores, joint pain, muscle pain, skin lesions which blister and ooze with sun exposure, hair loss. Light short periods. Nausea and vomiting lessened when I changed my diet, limited simple carbs and sugar. Taking Elmiron and an I.C. diet. sick when drinking alcohol. Weight loss.

8/28/2007- IC improving. ER visit for thirst, dry mouth, dizziness, weakness, achy, stiff painful neck, hives. WBC low 3.7, Neutrophil low 43 and Lymphocyte high 49. Read the book, adrenal fatigue, noticed I was experiencing symptoms described in the book. I began taking adrenal rebuilder and my periods returned to normal. Mother diagnosed with scleroderma.

End of 2007 –Seeing an acupuncturist. Notice intolerance to caffeine.

Summer 2008- Son began breaking out in hives from exposure to sunlight.

7/31/08 -Moderate arthritis in foot. Tubal ligation, noticed reaction to opiates caused urinary retention and bladder infection from taking one vicoden.

10/2008- Nausea is worse, increase in sensitivity to sugars, fatigue worse, strange fluttering feeling in my chest, stomach sensitivities to uncooked vegetables and nuts, moderate strep c, treated with 10 days penicillin. Headaches, fuzzy thinking, stiffness in neck, soreness in neck/lymph area, labs show positive ANA diffuse/speckled, low Neutrophils 42, high lymphocytes 48. Stopped taking adrenal rebuilder, I wanted to find out if it really made a difference because I was feeling so horrible.
E.R. visit because of pleurisy after drinking alcohol the night before, dry mouth, shortness of breath, light headedness, fuzzy thinking. Pain and stiffness spreading.

11/ 2008-Began managing muscle pain with acupressure massage, yoga and heat. Sometimes see things that aren’t there in my peripheral vision. Strep c is continuous. Cooking all veggies to digest them better. Developing small bruises, pain inside body when sit down, chest pains, lower back pain comes and goes, itchy skin/pin prickly feeling which travels around. Painful stiff joints in am. Pain in left arm/shoulder. Small cut took a long time to stop bleeding.
12/08 At onset of period felt extreme mental confusion, ability to think decreased, very poor memory. Bowel problems have ranged between constipation and diarrhea since onset of i.c., better since dietary changes. Waking up early in the am between 1 and 6. This sleep disturbance occurring off and on since beginning of i.c. When taking adrenal rebuilder symptoms had gotten better now that im not taking it, worse.
More sensitive to cold. Limited (but not completely omitted) wheat products and dairy from diet Itchy skin/pin prickly feeling a little better. Pain and tingling in left arm and twitching in fingers C4 test low-15.
Diagnosed with fibromyalgia Hydroxyzine seems to help with sleep.

ginger said...

history part 2

1/09 During short light period, cognitive abilities decreased again. Have been experiencing clumsiness-. Increased sensitivity to pain. Weight loss.

03/09 tonsil irritation/infection continues. nodules in back of throat, tonsils taken out (opted not to have opiates for pain after surgery) energy level better. A lump on back of tongue is growing.
Started back on adrenal rebuilder, periods became normal and memory and brain fuzziness improved. Began getting blister on skin when in the bath or shower.
Eliminated salicylate products on skin and some from diet. Began taking Mucinex (guaifenestin). Fibromyalgia symptoms gradually improving.

6/09 lump in throat big and painful. I pushed on it and it popped caused pain in ear and sinuses. Accidently took too much magnesium for a month and had diarrhea.

7/09- Eliminated all wheat/gluten products from diet. Over the next few months fibromyalgia symptoms gone! IC symptoms gone! Sleeping great! But gastro, sinus and alcohol intolerance worsening. No longer thirsty all the time. No longer feel sensitive to sugar. No more finger twitching. No more pin prick sensations. Not as sensitive to cold. Starting to gain weight back since stopped eating gluten. Stopped ic diet.

9/-7/09- Diagnosed with cecal diverticulitis. Nausea, diarrhea vomiting, it’s the same feeling I get when I drink alcohol (related to pancreas?). On rice only diet sinuses cleared up stomach feels better, blisters almost gone. But when other foods introduced sinus and gastro symptoms flair up, chest pain increases, fluttering feeling in my chest and blisters become larger and increase in number. Lost all the weight I had gained. Stool comes out with bright yellow thickened substance and orange stool for several weeks, the color lessening over time. Urine has a strange odor (or maybe it’s just that I can smell it because im super sensitive to smells now?)

9/09- continue taking two Elmiron a day (down from three), off of Mucinex. No longer on IC diet. Taking two adrenal rebuilder pills a day down from five.

9/16/09- Took a bath using salicylate products woke up the next morning with fibro and IC pain. Back on Mucinex, symptoms gone again.

9/17/09- Using Nasonex for sinus symptoms and Tagemant for gastro symptoms. started a very restricted diet. Arthritis in feet flared up.

9/30/09- Discontinued using tagemant because of sudden increases in heart rate, faintness and dizziness. It also caused my ic to flare up. Not sleeping well again. My chest hurts and is uncomfortable. Sometimes pleurisy like pain in left lung. I can still feel my heartbeat and it doesn’t feel right. Treated a vaginal yeast infection. Scheduled an appointment with allergy clinic November 12th. Having a hard time keeping weight on. Started working every other day at work.

10/01/09- saw the dr today he said the heart issues are not from the tagemant and that I should continue taking it. he listened to my heart and said that I have an extra heartbeat and that he can hear a fluttering valve. My chest and upper back ach my left arm hurts and im super tired.
Meds- elmiron 2-3 x’s a day, nasonex, adrenal rebuilder 2 x’s a day, chon. Glu. Joint supplements 2x’s a day, calcium 2x’s a day, digestive pearl once a day, digestive enzymes 1 at each meal, 1 hydroxizne at bedtime. Going to start back on tag…

Anonymous said...

Hi Kevin, thanks for your blog - it has given me some good info. I am in the process of being diagnosed with SM by Dr. Castells. I just found out that I have a positive with the C Kit 816 mutation. I am going back to Dr. C to look at treatment options. After looking on the internet, so far, I havent found any treatment options for the 816, but I read a study on Dasatanib, and that lead me to your blog. Do you know whether Dasatanib is available to others who are not part of the study? Or where the study is at this point? Any info would be helpful. My next appointment isnt for a month, and that is a long time to worry about this.


Kevin said...

Hi Lisa,

I’m not positive but I’m one of possibly two people still on the Dasatinib. It’s not a cure unfortunately but it has allowed me to live a more normal life. I still have to be very careful regarding what I eat, stress, heat and all of the other triggers related to SM. I don’t believe Dasatinib is available to anyone not on the study. Like any study I have to keep a detailed diary of day to day happenings while taking the medication. They have left it up to me if I wish to continue taking the Dasatinib I’ve asked to continue what other choice do I have? My old way of life or this life!

I’ve heard good things about Dr. Castells. I also have the C Kit mutation, do you go into shock? If I can be of further assistance please don’t hesitate to ask. I’m very happy that so many people find my blog to be of assistance, I had some great people help me 13 years ago it’s nice to give a little back.

Take care of yourself,

Anonymous said...

Kevin - thanks for the quick response. Yes - I do shock. I have gastro issues, then my hands and feet burn, then I go into shock - severe hypotension. I am currently on some H1 and H2 blockers, and started gastrocrom last month. Now with the 816 test result, Dr. C wants to look at further meds. Are you able to work? I am having a really hard time working more than a few hours a day with my flushing and dizziness. Thanks again, Lisa

Kevin said...


WOW you are on at the same time  I’ve been unable to work for many years now NO one will hire someone who may need a co-worker to help save their life. My old employer terminated me after I became ill back in 97. I applied for disability it took me 3 years and having to talk to a United States Magistrate to receive my benefits. I worked in a chemical plant for 15 years, first time I became sick I was dumping 50lb sacks of pure BHT (preservatives) in to a hopper that’s what triggered my masto. I’ve often asked myself If I thought I could return to work but this illness is to unpredictable. I have diarrhea constantly, I’m always sick to my stomach and a 100 more symptoms on a daily basis that will not allow me to go back to a 9 to 5 job.

How is your husband handling this? A good deal of my family thought I was imagining things when I first became sick.

Anonymous said...

Me again, my husband is an absolute saint. I worry how long this can last. He has taken on so much. He does pretty much all housework and feeds our boys. My diet is so messed up. I have gone lowfat vegetarian because my triggers include meat and fatty foods. However, I rarely have much appetite. A couple of my attacks were really scary for us, so I worry about his stress level. It helps to talk with others like you who have handled this for much longer. Most people get that I am sick. Sadley ER doctors and EMTs have been the most trouble, thinking I'm some dumb woman with a panic attack. Bleh. I sound so negative tonight. Lisa

Kevin said...


I’m glad you have someone who understands what is happening, that’s so important for you at this stage. The attacks are very scary indeed. If I were you I would have those EMT’s and ER Doctors contact a doctor who can educate them about SM. Also make sure your husband keeps them on the right track while you are possibly unconscious. Have you ever tried taking Benadryl before you have an attack? If you are like me you’ll feel your stomach start to severely cramp really fast and then the diarrhea kicks in, right when you start to feel the first bit like you are going to have a problem take the Benadryl. I use to buy the children’s over the counter Benadryl and drink it straight from the bottle I can remember drinking a whole bottle to stop from having an attack.

If you ever need to talk with someone, I would be glad to call and visit with you and your husband regarding our illness. My wife is a real trooper as well, I would be lost without her.

Susan L said...

Hi Kevin - Just wanted to check in. Got my tryptase back today - it went up to 19.1 - still not overly high but my dermatologist wants help now managing my case. Can I ask where/who you see for help in managing your case? My family practitioner isn't that interested in helping manage my care - she thinks its no big deal. So I am out looking for a new on, with no real insurance. Oh the joy....

I wanted to check in - hope to talk to you again soon.

Kevin said...


If you like I can give you a call sometime about how to find a doctor or atleast what I did so that I received the care that I needed when no one in my town had a clue how to treat me. Just email me if you like at: and I'll call you as soon as I can, give me a good time to call. I hope you are doing well.


staleci said...


I found your blog and I think I have a few questions. I am in the process of being seen by an allergist. Here are my symptoms: During an IBS bout, my toungue will feel thick, my face is red, my heart is racing, my hands are stiff and tingly. My regular doctor had no idea what it was so he referred me to the allergist. She ran the first set of blood work and my levels came back normal. The allergist gave me a prescription for an epi pen and and H1/H2 blockers. I have to wait on those for more tests. Since my initial blood work came back, I am wondering if this is what I have. Do my symptoms sound like Mastocytosis? She has given me another blood draw form to go in within 4-5 hours after my next IBS bout. Is it common for blood work to come back normal? I told my daughter I want to eat at a place that I know upsets my stomach so it will react and I can go in for blood work. Anh insight you can give would be helpful.


Anonymous said...

hi kevin i am australan when i read your blog i thought i was looking in a mirror i saved it so all my family could see what iam goning through it has tacken a load off my shoulders I to am a shocker i was diagnosed anaphylaxis to bees and wasps and given 41 injetions of venom 3 years later i was found to have systemic masto i now realise that i have had this for at least 20 years lots of qustions not to maney answers i am 53 years old

Katy said...

It's so comforting to see that others are experiencing similar battles with mastocytosis. I was recently diagnosed with cutaneous masto and am relieved to finally have a diagnosis I can begin to treat. Before my diagnosis, I was misdiagnosed with PLEVA. SO I was using worthless steroid creams and injections. The injections were helpful and seemed to control my puffed up angry spots, but the cream was annoying and thinned my skin out tremendously. I am only 25 and am really surprised to see how many "symptoms" I actually have. I didn't associate all of my "issues" as being intermingled. There is a dermatologist conference in Indy where Chicago/Indy Physicians are coming together and they asked me to come so they could all examine my interesting case! (Aren't we all so interesting)!!!! I am just very happy to see that you started this open ended communication for Q & A. It is so nice and comforting to hear about others experiences with SM and CM. I really hope to attend the conference in October because it's nice to have others who can understand and provide insight. Thanks! --Katy from Indy

Stuffie0912 said...


I saw you wrote "I worked in a chemical plant for 15 years, first time I became sick I was dumping 50lb sacks of pure BHT (preservatives) in to a hopper that’s what triggered my masto."

BHT is a salicylate.

Salicylates, sulfites, gluten are all known triggers for mast cell disorders.

I am still researching. I never heard of masto till 2 weeks ago I ran into it on a salicylate sensitivity forum.

I seem to fit the sysptoms, and I am researching more to find out what tests to request.
Waiting on endoscopic biopsy results right now.

Smiley said...

Good afternoon I am 41, married with 2 teen girls. I have had anaphylaxis since 2006 and just found out I have systemic mastocytosis. Do you have an email so we could chat or know of any support groups on line?
Thank you so much,

Smiley said...

Good afternoon I am 41, married with 2 teen girls. I have had anaphylaxis since 2006 and just found out I have systemic mastocytosis. Do you have an email so we could chat or know of any support groups on line?
Thank you so much,

Mike Morse said...

Hello, I'm a newbie here, but so happy to not feel completely alone! I can sum up my symptoms with one word, OUCH! Is it just me or does it feel utterly exhausting? I am in a full blown attack right now and have my EpiPen sitting right by my side. I think a lot of us here have been through the wringer...GI, cardio, neuro, dermatologist, etc....I'm not sure if the couple of dozen doctor visits it took to get diagnosed was not the cause of even more stress which cascades into worse attacks. Mentally, I feel better knowing what is going on, but it still doesn't take the 'OUCH' away. I live in New Mexico, so we're a small state that doesn't have very many docs who see it very often. It took a lot of persistence and I have extremely bad epilepsy to go a long with it. I'll tell you that the heat and friction from seizing on carpet causes it to become even worse.
Hang in there though, and listen to your body. If you think something is wrong.....It probably is! Don't get discouraged even though I know how much it hurts. Keep writing and blogging until you can't anymore. It sure helps me. Best of wishes and speedy help to you all.

Anonymous said...

Hi There,

I was wondering if you can update me with how you are doing?
I am now 32 years old, on all H1, H2 and Chemotherapy (Gleevac for 2 years everyday now.)
I was thought to have Cutaneous, and systemic MCAD since 2011; however, been very ill with my first hives at 6 mths old baby.
When diagnosed 2011/2012, I was immediately placed on all H1/2 blockers as over time I was not responding to the course of treatments, and episodic events 10-15 in 6-8 mths of unknown anaphylatic events. In Dec of 2012 bone marrow had less than 25 mast cells (15 present,) skin biopsy shown small amount of Mast Cell;Upper,lower Gi was done at the same time. It took 3 mths to get the results back, and in 10 (all) biospy of the lower Gi, high amounts of Mast cell infiltration.

As I stated earlier I am on all H1, H2, and Gleevac everyday, 2 years now. I have good days and bad. Most recently, over the last 8 month, my symtoms are getting worst by the day.
stomach, gastro pains are unbearable, headaches, neck, back of head pain/throbbing and pain through the skull into the face is getting worse! Back pain, spine, overall is getting bad. My mind is good, focused and want to go,go go, but I am soooo exhausted even on stimulants for fatigue. My tacycardia and fluttering of the heart with low BP is getting worse...
I live in Canada and the Drs are not knwn to this Rare Blood Disorder MCAD (cutaneous/systemic.)
My symptoms first got better the 1st year on Gleevac, but now, n longer, and getting more intense.... There is a long list of symtoms but is there any hope for me? Is this just a bad interval or is this slowly coming to an end??

Please help, I am scared, 32 years old, with a little girl (7 years old.) My Insurer wants to send me back to work, however, I am unable to continue just normal living now.

Thank you,

Desparate Canadian Mom

Smiley said...

I was on Gleevac for 4 months. I had every side effect and my daily living was unbearable. I told my doctor no more my quality of life was not worth it on Gleevac. I am all the blockers and nasal spray is choromlyn sodium. So much better now. I work full time and I am tire a lot in the evenings with leg pain off and on stomach issues.

Kevin said...

Hi Brandi, It's a tough road for sure! I still struggle daily with the simple things that trigger our illness. I still take my protocol daily trying not to let this illness get in my day to day! You can find me on facebook here: Sorry for the slow reply.

-=Peace Kevin

Information Sheet for Patients and Caregivers!

The Mastocytosis Society,Inc. Information Sheet for Patients and Caregivers

This is a brief introduction for new patients and doctors who are unfamiliar with the management of mastocytosis. The information presented here, combined with that available in the cited references, provides a starting point from which to approach understanding, treating, and living with this rare disorder.

Mast cell disease, or mastocytosis, is characterized by the proliferation and accumulation of mast cells in a variety of tissues and can affect either sex at any age. Definite diagnosis is made by demonstrating an abnormal accumulation of mast cells in a biopsy, usually of the skin and/or bone marrow. Other causes for symptoms should be ruled out, and blood and urine testing for mast cell products may be suggestive of the diagnosis. When performed properly by experienced personnel with access to current information on recommended protocols the results of these tests will be useful in diagnosing and evaluating mastocytosis.

Mast cells are widely distributed in nearly every organ of the body, mainly close to blood and lymph vessels, nerve endings, and skin and mucous membrane surfaces. They develop from immature cells produced in the bone marrow, which migrate to the tissues where they mature.

Mast cells produce various chemicals which normally serve protective, inflammatory and regulatory functions as they interact with white blood cells and tissues. In mastocytosis these chemicals, or mediators, are abnormally abundant and cause symptoms.

Mast cell products (mediators)

Some mast cell products are stored in granules within the cell, and others are produced in response to stimulation by the immune system or by drugs, chemicals, or physical factors. Below is a table of some factors which can cause mast cells to release their products. Stress, strong emotions and estrogen can increase their effect.

The products present in granules and ready for immediate release on stimulation include histamine, heparin, tryptase, and chymase. These chemicals cause, and to some extent regulate, allergic and inflammatory changes, and are involved in tissue building or repair. In response to immune system activation of the mast cell, arachidonic acid within the cell is converted into prostaglandin D2 and leukotriene C4, which restrict air flow in the lungs, stimulate mucous formation, and attract some kinds of white blood cells.

Mast cells also generate several cytokines, which are proteins that interact with white blood cells and tissue cells to continue the allergic or inflammatory response.


The symptoms of mastocytosis vary from person to person and may occur as "attacks" or as simply fatigue and a feeling of ill health. Over time, symptoms may become more frequent and more severe. The rate of progression differs from person to person, and there may be an improvement in symptoms for long periods of time. The type and severity of symptoms can also vary greatly from person to person or from one episode to the next. Often, seemingly unrelated symptoms comprise an individual's personal pattern of mastocytosis. These may (or may not) include: flushing (temporary skin redness), itching, hives,bruising, and skin sensations such as tingling. Other symptoms experienced by many of the people with mastocytosis are nausea,vomiting, abdominal cramping, occasional or frequent diarrhea, and excess stomach acid or ulcers. The person may experience unexplained fractures, mild to severe pain in bones, joints, or muscles, enlargement of liver or spleen, bladder pain, heart palpitations or rapid heart beat, chest discomfort, shortness of breath, light-headedness, fatigue,weakness, weight loss, respiratory symptoms including asthma. There may be depression, poor memory or irritability; also intolerance to heat or cold or to a change in temperature. Other symptoms include headaches,fainting or near fainting, and recurrent anaphylaxis.


Because there is not yet a cure for mastocytosis, treatment is aimed at reducing the frequency and severity of the release of mast cell products and at countering the effects of mast cell products which are inevitably released. Most patients will achieve relief of symptoms only by employing measures in both areas.

Avoidance of the factors which are known to cause a reaction for the individual patient, and cautious evaluation of other factors, such as those listed in the Table below, are important in the management of mastocytosis.

In using drugs to counter the effects of mast cell products,treatment must be tailored to the individual patient. Most commonly, H1 antihistamines such as chlorpheniramine or hydroxyzine are used to decrease the skin symptoms, vasodilation and mucous secreting actions of histamine. Stomach symptoms generally respond well to H2 antihistamines, such as cimetadine or ranitidine, which can also help reduce skin symptoms.

Aspirin or other non-steroidal anti-inflammatory drugs, if tolerated by the patient, provide relief from flushing and lightheadedness by blocking the body's production of prostaglandin D2. These drugs can cause unexpected severe reactions, though, and their use must be instituted cautiously under careful medical supervision.

Cromolyn (disodium cromoglycate) is a mast cell stabilizing drug which is frequently effective in reducing skin and gastrointestinal symptoms as well as mental and other systemic systems.

For severe diarrhea or malabsorption, collection of fluid in the abdominal cavity, and continued anaphylaxis which fails to respond to other measures, systemic corticosteroids may be required.

People with mastocytosis should carry injectable epinephrine, and they should know how to inject themselves if necessary to treat anaphylaxis.

There are other medications which may provide symptomatic relief if the ones mentioned above are ineffective or undesirable for a particular patient. No new medication or remedy should be started without careful consideration and close supervision, in case of an unexpected reaction.


It is not yet possible to predict the course of mastocytosis in any individual person. When involvement is limited to the skin, symptoms may improve or clear entirely, but it is also possible for the disease to progress to the systemic form. In about half the young children affected, symptoms disappear as they reach adulthood.

When the mast cell infiltration is systemic, symptoms may progress slowly over many years or may suddenly increase temporarily or permanently. The patient may even progress to the more serious categories of disease.

For the small percentage of patients who develop an associated hematological disorder, the course varies, and the prognosis depends on the associated hematological disease. ( 2 )

It is very important for the patient and the doctor to maintain good communication and to work cooperatively to achieve the best possible symptom control. Communication with other doctors caring for mastocytosis patients, and with other persons who have the disease, is important in order to maintain a support and information network. The affected person, or the child's parents, should strive to become educated about mastocytosis and to be aware of their individual needs and responses to triggering factors. A recognized medical warning device, such as a MedicAlert bracelet, should be worn, and extra caution is needed when undergoing dental work or surgery.

The Mastocytosis Society provides education and support to people with all forms of mast cell disease and their doctors, encourages research, and hopes to help find a cure for the disease. The Society also maintains a list of consultants who are available to advise professionals caring for a mastocytosis patient. Because of the rare nature of mast cell disease, we encourage doctors and patients to register with the Society in order to facilitate the communication and information exchange which will hasten the achievement of a cure.

Classification (adapted from (1) )

The following is a useful classification of the kinds of mastocytosis.

Cutaneous mastocytosis: Skin involvement only. This may include:

Urticaria pigmentosa: The typical rash of mastocytosis in the skin.

Solitary mastocytoma - a clump of mast cells restricted to a small area of the skin.

Diffuse cutaneous mastocytosis - skin involvement without urticaria pigmentosa or telangiectasia macularis eruptiva perstans (distinctive patches of discolored skin with small blood vessels on a reddened background. If the skin is heavily infiltrated with mast cells, the release of large amounts of mast cell products may cause systemic symptoms.

Systemic mastocytosis with or without skin involvement: Mast cell infiltration of at least one internal organ (like bone marrow or gastrointestinal tract). (2)

Mastocytosis in association with hematological disorder, with or without skin involvement: For example: leukemia, lymphoma, and myelodysplastic or myeloproliferative disorders.

Lymphadenopathic mastocytosis with eosinophilia, with or without skin involvement: Enlarged spleen and lymph nodes, infiltrated with mast cells, along with a blood count high in eosinophils.

Mast cell leukemia: This is rare but is the most serious form of masocytosis. The treatment and course of the disease is dictated by the leukemia.

Table of mast cell degranulators (adapted from (3) )

Allergens and other immunologic stimuli


Physical stimuli (heat, cold, sunlight, friction, pressure, vibration)

Bacterial toxins

Venoms (snake and insect, especially bee and wasp stings)

Biologic polypeptides (released by intestinal roundworms, jellyfish,crayfish, and lobster)

Polymers (Compound 48/80, dextran)

Drugs: Aspirin, alcohol, narcotics (codeine, morphine), polymyxin B,amphotericin B, D-tubocurarine, quinine, iodine-containing radiographic dyes, scopolamine, gallamine,decamethonium, reserpine.


1. Dean D. Metcalfe. Classification and Diagnosis of Mastocytosis:Current Status. J Invest Dermatol 96: 2S-4S, 1991.

2. Linda Golkar, Jeffrey D Bernhard. Seminar: Mastocytosis. Lancet 1997; 349:1379-85

3. Jack Longley, Thomas P. Duffy, Steven Kohn. Continuing Medical Education: The mast cell and mast cell disease. J Am Acad Dermatol 1995;32:545-61

Last update 6th December,2004