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Symptoms, Causes, Diagnosis, and Treatment

There are six types of systemic mastocytosis. Each type has different symptoms, but in general, the higher the abnormal mast cell accumulation, the greater the likelihood of serious health complications.
Indolent Systemic Mastocytosis (ISM)
Indolent (slower growing) systemic mastocytosis is the most common form of systemic mastocytosis in adults. People usually have milder symptoms that develop over many years. Skin and gastrointestinal issues are common, and usually other organs aren’t affected.
For people with ISM, the chances of progressing to more advanced disease are low — about 5 percent in a 10-year period, says Nikolai Podoltsev, MD, PhD, an associate professor of internal medicine specializing in hematology at Yale Medicine in New Haven, Connecticut.
Smoldering Systemic Mastocytosis (SSM)
SM With Associated Hematologic Neoplasm (SM-AHN)
Aggressive Systemic Mastocytosis (ASM)
In aggressive systemic mastocytosis, mast cells infiltrate organs such as the liver, spleen, bone marrow, lymph nodes, and small intestine.
Mast Cell Leukemia (MCL)
Mast Cell Sarcoma (MCS)
Indolent and smoldering SM are less aggressive forms of the disease, whereas SM-AHN, ASM, MCL, and MCS are all considered advanced systemic mastocytosis (advSM), says Dr. Podoltsev.