| Phenotype / cell stem origin | mast cell |
| Etiology | involvement of KIT/SCF has been demonstrated in a few cases, but the diversity of the clinical pattern has not yet been elucidated;increased soluble SCF has been reported in the skin of patient with indolent mastocytosis; c-KIT mutations have been identified in patients with all forms of sporadic mastocytosis. |
| Clinics | indolent mastocytosis involves the skin, bone marrow and gastrointestinal tract; clinical features range from a single cutaneous nodule to multiple pigmented macules resulting from increased epidermal melanin and papules (urticaria pigmentosa) or diffuse cutaneous involvement; bullae, vescicles and abnormal telangiectasia may be seen; gastrointestinal involvement leads to symptoms such as nausea, vomiting and abdominal pain. in mastocytosis with an associated hematological disorder the urticaria pigmentosa symptoms are accompanied by a variety of haematological findings due to mast cell infiltrates to bone marrow, spleen, liver and lymph nodes. mast cell leukemia is characterized by proliferation and infiltration of immature mast cells in bone marrow, peripheral blood and various extramedullary tissues. aggressive mastocytosis is characterized by aggressive involvement of several haematopoietic organs |
| Pathology | accumulation of mast cells in various organs and release of mast cell mediators which are responsible for the different clinical signs |
| Prognosis | highly dependent on the form being severe, often fatal, in all types with the exception of the indolent form |
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