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RESEARCH PRODUCT
Mastocytosis - pathogenesis, clinical manifestation and treatment
Petra StaubachNicola Wagnersubject
0301 basic medicinemedicine.medical_specialtyGastrointestinal tractbusiness.industryDermatologymedicine.diseaseMast cell leukemiaDermatologyMast cell proliferation03 medical and health sciences030104 developmental biologymedicine.anatomical_structuremedicineMast cell sarcomaHematological neoplasmAllergistsBone marrowbusinessAnaphylaxisdescription
The term mastocytosis designates a group of rare disorders characterized by typical skin lesions, frequently associated episodes of anaphylaxis, and clinical symptoms related to the release of various mediators. Dermatologists/allergists are frequently the first to establish the diagnosis. The condition is based on clonal mast cell proliferation, usually in the skin or bone marrow and only rarely in the gastrointestinal tract or other tissues. In general, mastocytosis has a good prognosis in terms of life expectancy. Rare variants - including mast cell leukemia, aggressive mastocytosis, and the exceedingly rare mast cell sarcoma - require cytoreductive therapy. In cases associated with hematological neoplasms, the prognosis depends on the underlying hematologic disorder.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2017-06-29 | JDDG: Journal der Deutschen Dermatologischen Gesellschaft |