Mastocytosis Systemic

Question: Please please tell me everything you know about systemic mastocytosis.? A friend of mine was just told she has systemic mastocytosis. What can you tell me about this disease? Do you know anyone who has it?

Answer: Systemic mastocytosis is caused when mast cells collect in the tissues and can affect organs such as the liver , spleen , lymph nodes , and bone marrow. Mastocytosis is a disease characterized by the presence of too many mast cells in various organs and tissues. Description The body has a variety of free-roaming cell populations that function as immunogenic agents. Most immunogenic cells fall into the category of white blood cells, but some remain in tissues and are not found in the blood. Mast cells are such a group. The cause of mastocytosis is unknown. People with systemic mastocytosis have bone and joint pain. Peptic ulcers are frequent because of the increased stomach acid stimulated by histamine. Many patients with systemic mastocytosis also develop urticaria pigmentosa. These skin lesions itch when stroked and may become fluid-filled. Systemic mastocytosis is only symptomatically treated. There is no known treatment that decreases the number of mast cells within tissue.

Question: Systemic Mastocytosis: Mortal or life-threatening? Hi there. Mi doctor believe that I have Mastocytosis, and Mastocytosis in an adult like me could mean: Systemic Mastocytosis. A lot of tests will have to be done to confirm the diagnosis but now I scared to dead: I surfed the web and in some webs they says that Systemic Mastocytosis is a mortal illness but in some others they says that systemic Mastocytosis have no cure but is a life-threatening condition. What is the true? Thank you, P Well. Doctor believes I have mastocytosis, he believes that my arms rash is mastocytosis. A skin biopsy was made and today they will give me the results. What scare me is that my hematology exams have never been normal: Since I was child I have leucopeny and hight-ferritine. A lot of exams have been made Through my life and nothing bad have been found (even in three bone marrow biopsies). I have lived 30 years of a normal life and no serious illness although my hematology condition, but today if a doctor says me that I have mastocytosis, maybe it could mean the beginning of the end for me.

Answer: Sorry to hear that this may be the case. I'm curious as to what signs you presented with that led your doctors to this diagnosis. Bottom line is that a bone marrow biopsy will need to be done (don't be afraid of this, it isn't that bad/painful) unless the disease is limited to the skin. Buuut you're saying it's possibly systemic so the bottom line is: 1.The disease is too rare to know a prognosis for sure but, 2.The prognosis is variable and depends on your age, degree of symptoms and spread of disease. 3.Unfortunately the median survival time is probably 2-6 yrs, but there isn't too much data. Hope this helps and best of luck. Edit:I know it's a difficult thing to do but try to relax until a definitive diagnosis is established. A bone marrow biopsy (or biopsy of other suspected organs) will need to be done before any plan can be decided. I don't think even the best dermatologist can diagnose mastocytosis just from looking at a rash, IMO. Again, good luck mate.

Question: Survival Rate of patients with systemic Mastocytosis? someone with this disease, mainly seen on the skin, however showed up in a bone marrow biopsy,......also had gull bladder removed, not sure if it was due to her recent childbirth,....or the disease,......being treated with chemo pills, at this time,....what is the survial rate, and how much "time' would this person have left in life?

Answer: Many people with systemic mastocytosis live long lives. did this person get it as a child or in adulthood? the odds are much better in children. Mastocytosis is not cancer by any means, although systemic masto is linked to lymphoma and leukemia. This person should be seeing a mastpcytosis specialist. there are several in the country. I know of one in Boston if you need contact info.

Question: Severe Stomach Pain & Systemic Mastocytosis?? Hi there... I've been diagnosed with systemic mastocytosis. I've had it under control for about 3 years now, but suddenly have had a huge flare-up that no one can explain. I've been sick since the first of the year with severe nausea, stomach pain and vertigo. While changing up my antihistimines has helped, I can't seem to get rid of the stomach pain, and am still experiencing some nausea! I'm taking 40 mg of Pepcid daily and I'm still suffering. Seems to happen anytime I eat, so I've pretty much stopped eating (I know, not good -- but what would YOU do if you were in agony every time you ate?). I know it's not an ulcer -- we've checked that. I know mastocytosis can cause stomach pains like this, but the Pepcid has always fixed it in the past. Does anyone else have mastocytosis? Any ideas for me?? I'm leaving for my honeymoon in 4 days and I REALLY don't want to be sick the whole time!!! Thank you for your help!

Answer: There is currently no cure for mastocytosis. However, there are a number of medicines to help treat the symptoms of mastocytosis: Antihistamines block receptors targeted by histamine released from mast cells. Both H1 and H2 blockers may be helpful. Leukotriene antagonists block receptors targeted by leukotrienes released from mast cells. Mast cell stabilizers help prevent mast cells from releasing their chemical contents. Cromolyn Sodium Oral Solution (Gastrocrom® / Cromoglicate) is the only medicine specifically approved by the U.S. FDA for the treatment of mastocytosis. Ketotifen is available in Canada and Europe, but is only available in the U.S. as ophthamic drops (Zaditor®). Proton pump inhibitors help reduce production of gastric acid, which is often increased in patients with mastocytosis. Excess gastric acid can harm the stomach, esophagus, and small intestine. Epinephrine constricts blood vessels and opens airways to maintain adequate circulation and ventilation when excessive mast cell degranulation has caused anaphylaxis. Albuterol and other beta-2 agonists open airways that can constrict in the presence of histamine. Corticosteroids can be used topically, inhaled, or systemically to reduce inflammation associated with mastocytosis. Immunotherapy (Allergy Shots) may rarely be beneficial to select patients with mastocytosis. The benefits of the shots should be weighed against the substantially increased risk of severe and potentially fatal anaphylaxis triggered by the shots themselves. Antidepressants are an important and often overlooked tool in the treatment of mastocytosis. The stress and physical discomfort of any chronic disease may increase the likelihood of a patient developing depression. Depression and other neurological symptoms have been noted in mastocytosis (see Rogers et al.). Some antidepressants such as doxepin are themselves potent antihistamines and can help relieve physical as well as cognitive symptoms. In rare cases in which mastocytosis is cancerous or associated with a blood disorder, the patient may have to use steroids and/or chemotherapy. The novel agent imatinib (Glivec® or Gleevec®) has been found to be effective in certain types of mastocytosis (Droogendijk et al.). There are clinical trials currently underway testing stem cell transplants as a form of treatment. There are support groups for persons suffering from mastocytosis. Involvement can be emotionally therapeutic for some patients.

Question: Yes are there any cures for mast cell disease ? also know as Mastocytosis,systemic mastocytosis,SMCD? My doctor says that in five years they can remove these mast cells with lasers, has it been done ?.. I THINK IT HAS IN MICE ? It would really improve my quality of living, it also could improve my dermatograpism & or Cfids. and or fibromyalgia, I heard my disorder is so rare only 2 people in every 33,000 have it ,any feed back would be greatly appreciated and respected..thank you

Answer: I was diagnosed with Systemic Mastocytosis 7 years ago. There are treatments to help with symptoms but there is no cure. Laser treatment can reduce skin lesions but it is not permanent and not a cure.

Question: Anyone in Australia and have Mastocytosis? Hello, we have just moved to Australia and looking to get in touch with anyone with mastocytosis or systemic mastocytosis. My son has systemic mastocytosis. J

Answer: Hi, We've just moved to Melbourne. My daughter (18yrs) is currently undergoing testing for Mastocytosis. There is a contact person in Western Australia, these are the contact details. Good luck! http://www.tmsforacure.org/patientcontacts/waustralia.shtml Western Australia Patient Contact Leonie O'Connor Western Australia, Australia sunland@supernova.agn.net.au Kind regards Jenny Herd

Question: Could I have Mastocytosis or MCAS? 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?

Answer: Hi....I was diagnosed with Systemic Mastocytosis in 2000 at N.I.H. in Bethesda, MD. following 2 skin biopsies, a 24 hr. urine for HISTAMINES, and a Bone Marrow Biopsy. I have both the Cutaneous and the Systemic Forms of this rare blood disorder, and, over the years, it has progressed to the point where I am now receiving IV Infusion of a Chemotherapy drug called CLADRIBINE. I have been treated at the Univ. of Michigan by Dr. Cem Akin(who originally diagnosed me at N.I.H.,) as well as at Mayo Clinic, Rochester, MN. I also have a contact at the Dept. of Leukemia, MD Anderson Cancer Center, Houston, TX. All that being said, your symptoms reflect many possible other sources...have you had your gall bladder checked? That could account for the diarhhea and stomach pain when you eat foods with a high fatty content. Have you had your Calcium and Parathyroid levels checked? Hyperparathyroidism can cause extreme bone pain, vomiting, diarrhea, and Gastrointestinal Reflux Disease. The large, itchy hives you get in the abdominal area are anybody's guess...but because they seem to only be in the stomach area, instead of all over, that probably rules out Cutaneous Mastocytosis. Hives can also arise from anxiety and stress, or an allergic reaction to SOMETHING, but again, an allergy that produces hives in only one area probably isn't due to an allergen like a new detergent, new body lotion, etc. As the previous source mentioned, a high Tryptase level is usually indicative of a MCAS. Get your Alkaline Phosphytase level checked..a high serum level might confirm that you are indeed having bone involvement and might be a starting point from which your dr. can correlate your unexplained bone pain....although you are very young, a DEXA Scan of your bones could show Osteopenia or Osteoporosis and Bone Fragility, but those scans are more often used in older women. It has taken me YEARS to find appropriate help and I know just how nerve wracking it can be to find out what's going on with you. How you ever even thought to think of any MCAS's tells me you're doing alot of online research, or that some Dr. at some time said something in an off handed manner about this group of disorders. If the latter is the case, GO BACK TO HIM!!! BTW, for your stomach cramps and diarrhea, see a Gastroenterologist and get a prescription for Gastrocrom...it really helped me! Also, when I was first diagnosed, all that was available to treat the symptoms were Histamine Blockers, such as Zantac, Zyrtec, Benadryl, and later Allegra, and Singulair. Also, real relief for my Reflux Disease as well as my Masto symptoms of the GI Tract, came in the form of Prevacid..it is a Proton Pump Inhibitor which, somewhere between 2000 and today became a STANDARD drug for treatment of Mastocytosis related GI problems. Sorry this was so long, but regardless of what is causing your symptoms, I wanted you to at least have some things to think about in your quest to find some answers!! Very best of luck to you!

Question: I was on a 120mg of prednisone and suddenly stopped it due to my doctor not refilling it. now i have hives...? my hives are getting worse and nothing is making them better. I also have a diagnosis of systemic mastocytosis and was totally off the prednisone for 5 days then restarted again.

Answer: Why on earth did your doctor stop the Prednisone? Did you miss too many appointments; did he go on vacation? Did he lose all sense he ever had?? If it's not your fault the prescription wasn't refilled, seek a new doctor and go ahead to the ER now so you can be put back on the Prednisone.

Question: Mastocytosis Progession? I am a 31yo Male who has been diagnosed via skin biopsy with mastocytosis. The only "syptoms" i currently posess are the UP or spots on my skin. My tryptase levels from blood tests were elevated but not over the 20 mark which is indicitive of the systemic form of the disease. I do have some acid reflux issues + slight asthma but these may or may not be associated with the condition. I want to know if it is likely or possible that my current condition, cutaneous, can progress to the systemic level, and if so, what can i do to help retard the progession. Additionally, is there anything to be done to stop the progression or development of more UP. Thanks for your input.

Answer: Cheer up! It's extremely rare for it go to any other organs. (Oddly, skin is an organ) Google mastocytosis, then click the second option. You'll feel much better. It also sounds as if it responds well to treatment, and there are ways to minimize outbreaks.

Question: Anyone's child have a mastocytoma? My child was diagnosed with a "mastocytoma". It is something that looks like a large raised bug bite on his leg. It turns out to be a tumor of "mast cells," or allergy cells. The dermatologist was not too concerned and seemed to believe it would probably go away. It is tough for me to find information on this condition, but I know that "mastocytosis," a more systemic version of the problem, is extremely injurious. Also, the mastocytomas have the potential to turn malignant. Does anyone have experience with this type of pediatric tumor, or have any resources in this area?

Answer: yahooanswersmom, I have cutaneous and systemic mastocytosis. The cutaneous form (which is what your child was diagnosed with) is just a collection of mast cells. It's not necessarily something to be worried about but my question is - who diagnosed this and what is their experience with the disease? If it was your regular pediatrician who has only heard of these things or had maybe one patient with the condition, I'd see a pediatric dermatologist and/or allergist. Also, there is a wonderful website www.mastokids.org that is focused on children with mast cell conditions/disorders, like mastocytoma. If you have other questions - you can email me through my blog www.talesfromthestirrups.blogspot.com.