What do you know about systemic mastocytosis.?

See below. Systemic mastocytosis, often termed systemic mast cell disease (smcd), is a heterogeneous clonal disorder of the mast cell and its precursor cells. It is now classified as a myeloproliferative neoplasm. It is characterized by mast cell infiltration of extracutaneous (not skin) organs.

Related Questions

What is done for systemic mastocytosis?

Control of symptoms. After diagnosis, treatment is mostly focused on controlling symptoms. Flushing, itching, and (to some extent) GI symptoms are helped by antihistamines +/- leukotriene antagonsists (e.g. Monteleukast). More severe GI symptoms are treated with cromolyn. Cromolyn mixed with lotion can sometimes help severe itching. Epipen (epinephrine) is used for anaphylactic episodes. See a specialist in masto for more info.

Help! I have aggressive systemic mastocytosis. What do I do?

See a specialist! Treatment of aggressive systemic mastocytosis can be extremely complex. If at all possible, you should see a specialist with experience treating this disorder. The mastocytosis society maintains a list of specialists, which can be viewed at http://tmsforacure. Org/patients/research_centers_1.Php. There are ongoing clinical trials for treatments that may help slow the progression of the disease.

Please please tell me everything about systemic mastocytosis.?

Skin problem. This is a skin disorder. I recommend asking a dermatologist this question.
See a specialist. Systemic mastocytosis (sm) is an overgrowth of mast cells (immune cells that release histamine and other chemicals upon activation) in the bone marrow and throughout the body. Symptoms vary from flushing to hive-like reactions to recurrent anaphylaxis. Sm can present in many different ways and have different treatments. See an allergist or immunologist with expertise in sm for more details.

How much time does the average person with systemic mastocytosis live?

Widely variable. Overall about 60% are dead in ten years, 80% on 20 years but 10% are long-term survivors; there are no cures. A lot depends on the genetics of your lesion. Here's the best article http://bloodjournal. Hematologylibrary. Org/content/113/23/5727? sso-checked=1.

Survival rate with systemic mastocytosis?

Normal. Unless there is an anaphylactoid reaction there should be a normal life span.
Usually normal. Most people with a systemic mastocytosis have the 'indolent' variey, in which case their life span is the same as anyone else's (provided they get appropriate treatment for any anaphylactic reactions). There are very rare cases that we call 'aggressive.' in this case, it may shorten expected lifespan depending on just how 'aggressive' it is, but some of these may be amenable to treatment.

Is systemic mastocytosis life-threatening?

Usually not. Most people with systemic mastocytosis (sm) have a normal lifespan. Episodes of anaphylaxis with sm can be dangerous if not treated properly, so it's important to use Epipen (epinephrine) and lay flat with the feet elevated slightly. Rarely, sm can be aggressive or associated with a leukemia. In these cases, lifespan may be shortened, but the sm may respond to chemo. See a specialist in masto for more info.

What is the survival rate of patients with systemic mastocytosis?

Usually normal. Most people with a systemic mastocytosis have the 'indolent' variey, in which case their life span is the same as anyone else's (provided they get appropriate treatment for any anaphylactic reactions). There are very rare cases that we call 'aggressive.' in this case, it may shorten expected lifespan depending on just how 'aggressive' it is, but some of these may be amenable to treatment.

Is treatment necessary for systemic mastocytosis? I am taking gleevec (imatinib). What would happen if I stopped taking it?

D/w oncologist. There are indolent systemic masto, and agressive systemic mastocytosis (asm). Many times, with indolent disease- therapy will be aimed only to control the symptoms- itching, diarrhea etc. If you have an asm- then usually treatment is started. Asm can manifest from slowly progressive to rapid fatal disease. Organ involvement, transformation to other blood DX including acute leukemia can happen.