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| Mast cell disease | Subtype | Description | Major risks |
| Cutaneous mastocytosis | Maculopapular cutaneous mastocytosis (MCPM), usually called urticaria pigmentosa (UP), includes telangiectasia macularis eruptiva perstans (TMEP) | Too many mast cells in the skin
The most common presentation of mastocytosis Red/brown macules (UP), occurs in adults and children Spider veins associated with mast cells (TMEP), almost exclusively in adults Mast cells may be extremely reactive throughout the body |
Anaphylaxis |
| Cutaneous mastocytosis | Diffuse cutaneous mastocytosis (DCM) | Too many mast cells in the skin
Redness, blistering, thickening of skin Almost exclusively occurs in children Mast cells may be extremely reactive throughout the body |
Damage to skin
Anaphylaxis |
| Cutaneous mastocytosis | Solitary mastocytoma of the skin | A benign mast cell tumor on the skin
Redness and swelling when touched (Darier’s Sign) Despite name, multiple mastocytomas are sometimes found on the skin Despite name, mastocytomas can also occur in other parts of the body, including GI tract Mast cells may be extremely reactive throughout the body |
Anaphylaxis |
| Systemic mastocytosis | Indolent systemic mastocytosis | Too many mast cells in an organ that is not the skin
Initiated in bone marrow Most common form of systemic mastocytosis Symptoms in multiple organ systems Benign Normal lifespan Mast cells may be extremely reactive throughout the body |
Anaphylaxis |
| Systemic mastocytosis | Smoldering systemic mastocytosis | Too many mast cells in an organ that is not the skin
Initiated in bone marrow Symptoms in multiple organ systems Shows signs of progression toward malignancy Shortened lifespan Mast cells may be extremely reactive throughout the body |
Progression to a malignant form of SM like ASM or MCL
Progression toward organ damage Anaphylaxis
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| Systemic mastocytosis | Aggressive systemic mastocytosis | Too many mast cells in an organ that is not the skin
Initiated in bone marrow Symptoms in multiple organ systems Malignant Shortened lifespan Mast cells may be extremely reactive throughout the body |
Progressive organ damage
Anaphylaxis |
| Systemic mastocytosis | Mast cell leukemia | Too many mast cells in an organ that is not the skin
Initiated in bone marrow Symptoms in multiple organ systems Malignant Significantly shortened lifespan Mast cells may be extremely reactive throughout the body |
Rapidly progressing organ damage
Anaphylaxis |
| Systemic mastocytosis | Systemic mastocytosis with associated hematologic disorder | Too many mast cells in an organ that is not the skin
Initiated in bone marrow Symptoms in multiple organ systems Can be benign or malignant Lifespan dependent upon subtype of SM and other hematologic disorder Mast cells may be extremely reactive throughout the body |
Progression to a malignant form of SM like ASM or MCL
Progression toward organ damage Anaphylaxis |
| Mast cell sarcoma | Mast cell sarcoma | A highly malignant mast cell tumor
Symptoms in multiple organ systems Patient rapidly develops mast cell leukemia Malignant Significantly shortened lifespan Mast cells may be extremely reactive throughout the body |
Progression to MCL
Rapidly progressing organ damage Anaphylaxis |
| Mast cell activation syndrome | Monoclonal mast cell activation syndrome | Mast cells show signs associated with excessive proliferation
Considered by many to be a very early form of SM or a “pre-SM” Symptoms in multiple organ systems Mast cells are extremely reactive throughout the body |
Anaphylaxis |
| Mast cell activation syndrome | Mast cell activation syndrome | Mast cells do not show signs of excessive proliferation
Symptoms in multiple organ systems Mast cells are extremely reactive throughout the body |
Anaphylaxis |