Mast cell diseases

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

 

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